What diseases does a hematologist treat and their symptoms? What diseases are called hematological, and who treats them?

It is the hematologist studies factors that influence blood performance by certain organs and the ratio of its components, that is, hemoglobin, leukocytes, platelets, erythrocytes. It should be noted that the hematopoietic organs in the human body are the red bone marrow, spleen and liver. Various diseases, injuries can disrupt the process of hematopoiesis, so hematology works very closely with other areas of medicine, namely with therapy, surgery, traumatology, angiology and cardiology.

The goal of a hematologist is to treat patients with characteristic diseases and work with biological material in the form of blood and bone marrow. They also practice in laboratories to differentiate diagnoses by evaluating blood test results with special hematological tests.

What kind of doctor is this who specializes so widely in almost all areas of medicine? The answer is very simple and it lies in the fact that without blood the human body cannot exist, that is, it is food, breathing and the main purifier for it. In turn, this biological fluid cannot be complete if the main organs are damaged or missing. It is this relationship that makes the hematologist omnipresent in medicine, and the normal functioning of the whole organism depends on the composition of the blood and the work of the hematopoietic and circulatory organs.

Factors predisposing to the occurrence of blood diseases

Pathological changes in blood composition can be observed at certain points in a person’s life. In some cases, the indicators return to normal on their own, that is, the body copes with the problem, but more often you have to find out who the hematologist is. Here are the factors that can trigger the disease:

massive or mild but prolonged bleeding;
destructive and degenerative processes of red bone marrow of various etiologies;
bone injuries;
surgical interventions in the body;
lack of vitamins and minerals necessary for the normal process of hematopoiesis (vitamin B 12, iron, folic acid), usually due to poor and irrational nutrition;
pregnancy, childbirth, lactation;
Pathologies of the blood and hematopoietic system are inherited in 50% of cases.

The presence of such situations or physiological conditions should be a reason for an annual examination, even without obvious subjective reasons, that is, symptoms. It will be much easier for the doctor and the patient to exclude hematological diseases than to treat it. You must make an appointment with a hematologist in advance.

Common blood diseases

Statistics show that 10% of common diseases are somehow related to pathological changes blood, which explains the widespread introduction and development of hematology, as the most popular among areas in medicine.

Experts distinguish several types of blood-related pathologies:

general diseases that hematologists treat include the diagnosis of anemia of various etiologies (including in pregnant women), coagulopathy, platelet purpura, hemophilia, autoimmune blood disorders;
diseases of the blood and hematopoietic organs associated with oncology occupy a decent amount of space in this area, that is, we are talking about lymphomas, myeloid leukemia, multiple myeloma, macroglobulinemia.

In hematology, the doctor must be very competent and scrupulous, especially if the disease is diagnosed in a child.

Diagnostic methods in hematologist practice

When examining a patient, the hematologist carefully questions him for subjective complaints and palpates all accessible lymph nodes. The data obtained from such an examination determine the further plan for examining the sick person. Usually in hematological practice the following diagnostic methods are used:

Ultrasound of the abdominal organs and lymph nodes of the same department;
detailed general analyses;
taking biopsy tests from lymph nodes;
magnetic resonance imaging of the spinal cord, bones and internal organs;
blood test for INR, APTT and coagulogram;
sternal puncture, which involves further examination of the bone marrow.

The research results will help the doctor accurately establish a diagnosis for further development of a treatment plan. In adults, a hematologist can treat many types of diseases. In pediatric practice, such problems are dealt with by hematologists along with pediatricians.

Among doctors of various specialties there is a specialty of hematologist. Children are referred to a hematologist if there is a suspicion of diseases of the blood system.

WHAT IS THE BLOOD SYSTEM AND WHO IS A HEMATOLOGIST

There is probably no person who has not seen what blood looks like. This is a dark red liquid that appears immediately after a cut or more serious injury. At first, the blood is released in drops or flows out in a stream, and then it becomes thick, dense, and the bleeding stops. We say in such cases - the blood has clotted.

But blood is not just red water. It has amazing properties. And one of them is the ability to stop bleeding with the participation of special proteins of the blood coagulation system.

Another feature of blood, unlike all other organs and systems of the body, is that blood is in constant motion, and this movement is strictly ordered. It's like one way streets. In one direction, to the tissues, blood flows through the arteries, supplying oxygen and necessary nutrients there; in the other, through the veins, carbon dioxide and other waste products of cells are carried away from the tissues, which are then excreted from the body through the lungs, kidneys, intestines, and skin .

Thanks to the smallest branching vessels - capillaries, blood washes every cell and is the connecting link between all organs and tissues of the human body. It is not for nothing that this entire vascular system, and its length in an adult is more than 100 thousand kilometers, is called the “river of life.”

The liquid part of the blood is called plasma. Plasma is a real treasure nutrients. It contains proteins, fats, carbohydrates, vitamins, enzymes, hormones and minimum quantities almost all known chemical elements Mendeleev's system. They are called microelements. Some of them: iron, copper, nickel, cobalt - are directly involved in the process of hematopoiesis - the formation of blood cells.

And now I want to tell you about these amazing cells.

If we look at blood under a microscope, we will see that it contains many different cells. To do this, from a drop of blood taken from a finger, you need to prepare a thin smear on glass, paint it with special paints, and the microscope will provide high magnification and will allow you to see them clearly.

Blood under a microscope

Red blood cells

Look, almost the entire visible field is dotted with many pink “plates”.

These cells are called red blood cells. The hemoglobin protein they contain gives them their pink color. It is red blood cells that ensure tissue respiration. They transport oxygen and carbon dioxide using a special protein called hemoglobin. And if there are few red blood cells, and this happens with some blood diseases, oxygen starvation of the tissues develops, from which the entire body suffers.

And that's not all that red blood cells are capable of. It is known that different people there are different groups blood, positive or negative Rh factor. So, it is red blood cells that carry information about the group and rhesus affiliation of a person’s blood.

You probably know your blood type, the blood type of your children and loved ones. There are only four of them, and they are designated as 0(1), A(P), V(Sh) and AB(1U). It is important to know your Rh factor, especially for women. If a woman has negative Rh factor a child will be born with positive factor inherited from the father, their blood may be incompatible, and the child will develop the so-called hemolytic disease of the newborn. This occurs when Rh-positive red blood cells of the child enter the body of a Rh-negative mother through the placenta. The immune system the mother perceives them as foreign and develops antibodies against them. This danger increases after repeated births or abortion, since with each pregnancy the number of such anti-Rhesus antibodies increases.
You also need to know your blood type and Rh factor because now many people are offered to become blood donors for their loved ones if they are seriously ill and need blood transfusions.

The discovery of blood groups and the Rh factor can be considered a revolution in hematology. It made human-to-human blood transfusions possible and safe. It is only necessary that the blood of these people be compatible, and this can be easily determined by the doctor immediately before the transfusion. It was thanks to this discovery that we learned to save children from hemolytic disease newborns using exchange blood transfusions.

Leukocytes

Here large cells. Inside them there is a core, which consists of several segments, and fine grain is scattered around. These cells are called granulocytes, or neutrophils. And here are some smaller cells. They have a round nucleus that occupies almost the entire cell. This is a lymphocyte. And cells with a bean-shaped nucleus are called monocytes. And all together: neutrophils, lymphocytes and monocytes are defined in one word - blood leukocytes.
The role of leukocytes is difficult to overestimate. They protect the body from infections and other harmful effects, including providing anti-cancer immunity. At the same time, their functions are strictly delimited. For example, some types of lymphocytes, they are called T-lymphocytes, provide recognition of foreign cells and even remember what various microbes look like, others - B-lymphocytes, produce antibodies against them. And neutrophils and macrophages (macrophages are the same monocytes, but they work directly in tissues) “devour” them. And in this struggle, not only microbes die, but also the protective cells themselves.

Platelets

But in the blood smear there were still unnamed clusters of small dots. These are platelets. They are the first to enter into the process of stopping bleeding.

Red blood cells, white blood cells and platelets, or shaped elements, suspended in the liquid part of the blood - plasma and together with it form an important component of the blood system - peripheral blood.

Bone marrow. But the main organ of the blood system is the bone marrow, in which blood cells are born and mature. It is located deep in the bones.

Before entering the peripheral blood, cells pass through difficult process development. First they divide, resulting in two identical cells. And from these two cells four are then formed, from four - eight, etc. Then a long process of maturation occurs, and only after that the cells leave the bone marrow. At first, the blood cells in the bone marrow are similar to each other, and only then, having gone through the entire development path, having received “education”, they become the erythrocytes, leukocytes and platelets already known to us.

Bone marrow

How does the process of cell division take place? At the head of this entire system is the ancestral stem cell, which, under the influence of special growth factors, can differentiate in various directions. One of its sprouts produces red blood cells, another produces neutrophils, and the third produces platelets. Lymphocytes and monocytes go through their development path.

Cell division

The functioning of the bone marrow as a hematopoietic organ begins very early, from the end of the third month of the child’s intrauterine life, and before that, blood cells are formed in the liver, spleen and lymph nodes. These organs are then called organs of facultative hematopoiesis, that is, not obligatory, but potentially possible. This is important to know, because with different pathological conditions in children, a return to this embryonic type of hematopoiesis easily occurs, which explains the enlargement of these organs in many diseases, especially cancer.

In a healthy person, the bone marrow produces such a number of cells that it is enough not only to cover immediate needs. Thus, it contains 10 times more neutrophils than in the bloodstream, and a three-day supply of young red blood cells. Therefore, at different pathological situations the bone marrow can immediately respond with an increased flow of cells into the blood.

As we can see, hematopoiesis is a dynamic system that quickly responds to any influences to which the body is exposed. This often leads to diseases of the blood system itself.

Blood cells live relatively short and die after completing their work. They are replaced by new cells from the bone marrow, ready to immediately begin performing their duties. This process happens constantly. Red blood cells live the longest in the blood - 120 days, platelets - 10 days, and neutrophils - only 10 hours.

Spleen

There is also a special organ in the body in which old, spent cells are destroyed. This organ is called the spleen.

But the spleen has the ability to capture and destroy not only aged cells. In a number of diseases, it intensively captures and destroys blood cells that have changed during the course of the disease or are loaded with antibodies.

Thus, the blood system is the interaction of its three main components: bone marrow, peripheral blood and spleen.

When this well-functioning system is disrupted, diseases develop that are classified as diseases of the blood system. They can occur when the bone marrow or peripheral blood cells are damaged.

The science that deals with blood diseases is called hematology, and the doctor who treats patients with diseases of the blood system is called a hematologist, from the Greek word haima - blood.

WHAT COMPLAINTS DO YOU APPLY TO A HEMATOLOGIST?

Blood diseases often cause general malaise, weakness, fatigue, and loss of appetite. And often these complaints can be the first signs of very serious diseases that are associated with a violation of the main function of the bone marrow - hematopoiesis. Therefore, changes in the child’s condition and behavior should definitely be a reason to consult a doctor.

The subject of particular concern for parents is usually the pallor of the child’s skin, sometimes with a jaundiced tint, the appearance of bruises, nosebleeds and other bleeding.
With blood diseases, children may also complain of pain in the bones, joints, and spine. Sometimes headaches and stomach pain bother me.

They often consult a hematologist about an increase in lymph nodes. Lymph nodes perform important protective functions in the body. They stand in the way of the possible spread of the disease and often cope with it on their own, through local inflammation. This manifests itself in enlargement and soreness of nearby lymph nodes.

The local inflammatory reaction when encountering an infection manifests itself especially violently in preschool children. In children under two years of age this barrier function lymph nodes are underdeveloped. Infectious agents pass through such a node into the blood without hindrance, the infection “disperses” throughout the body, and the disease often takes on a generalized (widespread) character.

In older children and adults, suppression of infection in the lymph nodes often goes unnoticed and is asymptomatic.

Typically, regional (closest to the site of infection) lymph nodes become enlarged. For example, enlargement of the submandibular glands due to infection in the mouth and throat (carious teeth, stomatitis, sore throat). With cat scratch disease, the lymph node closest to the scratch site becomes enlarged. For some childhood infections, such as measles, rubella, infectious mononucleosis and other viral infections are characterized by an increase in the posterior cervical nodes.

And for a hematologist and oncologist, it is important that the lymph nodes - as organs of facultative hematopoiesis - have favorable conditions for the development of tumor diseases such as leukemia, lymphogranulomatosis and other types of malignant lymphomas.
Therefore, enlargement of lymph nodes, especially in atypical places not associated with specific foci of inflammation, requires excluding the oncological nature of the disease.
The simultaneous enlargement of the lymph nodes, liver and spleen deserves special attention, which is characteristic, for example, of acute leukemia. Sometimes enlarged organs reach such large sizes that they can be discovered by the mother when changing clothes or bathing the child.

And if the central nervous system, then headache, vomiting, signs of irritation appear meninges- meningitis. This also happens with acute leukemia.

WHAT RESEARCHES DOES A HEMATOLOGIST CONDUCT?

If a blood disease is suspected, the doctor refers the child to a hematologist for consultation or hospitalizes him in a special hematology clinic. After examining the patient, the hematologist conducts special hematological studies, which give very important information about various pathological conditions.

Diagnostic value of blood test

Surely you or your children have ever had a blood test done and been anxiously awaiting the answer. Blood tests present indicators that reflect the final result of the work of all hematopoiesis in the bone marrow. We evaluate these results by the content of red blood cells, white blood cells and platelets in the blood.

Red blood indicators are red blood cells and the protein hemoglobin they contain. Healthy people should have at least 4 million red blood cells and at least 120 hemoglobin units. The hemoglobin content in one red blood cell determines color index. It can be low (hypochromic), normal and high (hyperchromic). With a decrease in red blood counts, anemia develops, which can be normo-, hypo- and hyperchromic, respectively.
Normal content leukocytes - from 4 to 9 thousand. In various pathological conditions, the number of leukocytes increases or decreases, and then we say that the patient has leukocytosis, or leukopenia.

They study the blood smear leukocyte formula, that is, the percentage of neutrophils, lymphocytes and monocytes. The leukocyte formula in children, unlike adults, has age-related characteristics. For example, in a child of the first year of life, the percentage of cells is the opposite of the formula for an adult. Further, these indicators change, and only by the age of 12-14 do they approach the values of adults, that is, neutrophils make up 60-70%, lymphocytes - 20-30% and monocytes - 5-10%.

The blood formula also contains single cells called eosinophils and basophils. They are involved in allergic reactions of the body.

The platelet count does not change with age and should be at least 200 thousand.
Various changes in the blood have corresponding clinical manifestations.
If the skin is pale, the doctor Special attention draws attention to a decrease in the level of hemoglobin, red blood cells and the nature of the color indicator.

In patients with severe infectious diseases, the number of leukocytes increases, and cells that have not fully undergone the maturation process appear in the blood formula. Such changes in the blood occur in purulent-septic diseases and are called leukemoid reaction. With viral infections, on the contrary, the number of leukocytes and neutrophils decreases, and with leukemia, young “blast” cells appear in the blood smear, which do not appear in blood tests in healthy people.

When the level of platelets in the blood decreases, bruises and petechiae appear ( pinpoint hemorrhages), bleeding. Sometimes bleeding occurs due to a decrease in the level of proteins in the blood coagulation system, as happens in hemophilia. Then spend special study blood, which is called a coagulogram.

In what cases is it necessary to perform a bone marrow puncture?

If there is a suspicion of blood cancer, a bone marrow examination is required. To do this, a bone marrow puncture is performed with a special thin needle, piercing the bone in the sternum area or ilium. The resulting drop of bone marrow is examined according to many parameters, which makes it possible to determine accurate diagnosis and prescribe appropriate treatment.

First, the doctor examines the bone marrow under a microscope, and does this in the same way as when examining a peripheral blood smear. However, unlike blood, where only mature cellular elements are present, all types of cells are represented in the bone marrow - both very young, and those that are maturing, and mature. In this case, the cells that develop towards erythrocytes, leukocytes, and platelets are clearly visible.

If the cellular composition of the bone marrow is normal, no additional research not required. But if there are a lot of young blast cells in the bone marrow, as happens with leukemia, or the bone marrow is devastated, as happens with suppressed hematopoiesis, then special, additional studies are required.

Sometimes a small piece of bone is taken for analysis and the structure and relationship of individual elements are studied directly in specially prepared preparations. bone tissue.
Today, ultrasound diagnostic methods (ultrasound) are also successfully used in the diagnosis of blood diseases. computed tomography(CT), nuclear magnetic resonance (NMR). These modern studies make it possible to determine the size and structure of organs, various bone disorders, and identify tumor formations various localizations.

WHAT DISEASES DOES A HEMATOLOGIST TREAT?

Anemia

Iron deficiency anemia

Most frequent illnesses blood systems are anemic. We are talking about anemia when red blood levels - hemoglobin and red blood cells - decrease. The concept of anemia includes diseases of different nature, but most often they are associated with iron deficiency and are therefore called iron deficiency anemia.

Iron metabolism plays an important role in the construction of hemoglobin in maturing bone marrow red blood cells. Iron is found in muscles (myoglobin protein) and is part of cellular respiratory enzymes. With a lack of iron in the body, many metabolic processes.

Iron deficiency anemia most often develops in children in the first two years of life. There are many reasons for this. Some depend on the conditions of intrauterine development, others on the developmental characteristics of children in the first year of life. Thus, a child may not receive enough iron even before birth: with severe toxicosis of pregnancy, with chronic diseases in the mother, especially if the mother herself suffers from iron deficiency anemia or is on a vegetarian diet. Twins, triplets and premature babies will also receive less iron per kilogram of weight, since iron reaches the baby in the last three months of pregnancy.
After birth, especially in the first year of life, the child grows rapidly. Over the course of a year, his weight triples, and his blood volume increases 2.5 times. Accordingly, the need for iron increases sharply. And this is where the biggest difficulties arise.

Iron enters the body only from the outside - with food. Different foods contain different amounts of iron, and, unfortunately, milk contains the least amount of it. And if we remember that milk is the main food in the first year of life, it becomes clear why children of this particular age group so often suffer from iron deficiency anemia.

Fortunately, at this age, children are constantly with their parents and are observed quite regularly by a doctor in a children's clinic. Therefore, changes in the behavior, condition of the child, and the appearance of pallor of the skin in most cases are detected in a timely manner and are successfully treated with iron supplements. At the same time, it is very important to establish a normal diet, introduce complementary foods, juices, and vitamin-enriched mixtures in a timely manner. Properly replenished iron deficiency leads to the child's recovery from anemia.
There is another group of children most susceptible to iron deficiency anemia - girls of puberty (puberty). At this time, a rapid restructuring of the body also occurs and the need for iron increases sharply. The diagnosis in these patients is usually made late. The disease accumulates over a long period of time and manages to develop the whole complex of symptoms characteristic of chronic sideropenia (the so-called iron deficiency). This is first of all fast fatiguability, drowsiness, sudden deterioration and perversion of appetite. Patients cannot stand the taste and smell of meat, fish and chew chalk and cereals with pleasure. Their skin is dry, their hair and nails are dull and brittle. Significant changes are noted in the mucous membranes of the mouth and pharynx, including disruption of the act of swallowing.

In addition, menstruation begins at this age, and in girls with any platelet disorders, they can take on a long, heavy bleeding. In this situation, iron deficiency anemia also develops due to loss of iron.

Iron deficiency anemia also occurs with prolonged bleeding in small portions that are not visible to the naked eye. Such blood loss most often occurs in the mucous membrane gastrointestinal tract at various diseases such as hernia hiatus diaphragms, polyps, vascular tumors(hemangiomas), ulcer duodenum and etc.

A necessary condition The recovery of patients in whom iron deficiency has developed as a result of chronic blood loss is treatment of the underlying disease that was the cause of the bleeding, and often through surgery. Only after these measures is it possible to restore iron levels and recover from anemia.

Hemolytic anemia

The second most common group of anemias is the so-called hemolytic anemia. In these diseases, the bone marrow produces a sufficient number of red blood cells, but due to various reasons they have a short lifespan and are quickly destroyed.

Almost all hemolytic anemias are congenital and hereditary. However, heredity varies. Each trait in the body is controlled by two genes: one of them is major, dominant, the other is minor, recessive. A disease is considered dominant in which one gene is diseased, but it is the main one and determines the disease, and the other is recessive, the minor one is healthy. A disease is considered recessive if it is controlled by two recessive genes, and both of them are “sick” and carry the same pathological information. Diseases with recessive inheritance are more severe and have a serious prognosis.
Among hereditary hemolytic anemias, the most common is microspherocytic hemolytic anemia of Minkowski-Choffard. It is inherited in a dominant manner, and the genetic defect is located in the erythrocyte membrane.

Much less common are the so-called espherocytic hemolytic anemias, which are inherited by recessive type and are difficult.

IN in this case The cause of accelerated cell destruction is a congenital deficiency of one of the many enzymes contained in red blood cells.

As a result of the described disorders, red blood cells in hemolytic anemia, as we said above, have a shortened life expectancy. With Minkowski-Choffard anemia, they are destroyed in the spleen, that is, in the organ where old, spent cells are destroyed and in normal conditions. In non-spherocytic hemolytic anemias, hemolysis (cell destruction) occurs wherever there are macrophages capable of trapping altered red blood cells, for example, in the liver, bone marrow and other organs.

The main clinical signs for all types of hemolytic anemia are pale skin, jaundice and an enlarged spleen. Such patients are constantly moderately pale and yellow, but periodically their condition worsens sharply, the temperature rises, and the pallor and jaundice of the skin increases. These are signs of exacerbation of the disease, so-called hemolytic crises. During this period, patients need special treatment measures, and often blood transfusions.

Even if Minkowski-Choffard anemia is mild and crises are rare, over time such a severe complication develops as cholelithiasis. Removal of the spleen leads to recovery in all patients. Blood tests normalize, jaundice disappears, and the formation of gallstones stops. However, the genetic defect remains, which is inherited. And even parents who have undergone surgery have high probability give birth to a child with hemolytic anemia. But they already know about the miraculous effects of splenectomy.

This cannot be said about non-spherocytic hemolytic anemias. Fortunately, they are rare. Since the destruction of red blood cells occurs in many organs, removal of the spleen gives a partial effect or no effect at all.

We have become acquainted with such anemias, the origin of which is not directly related to dysfunction of the bone marrow.

In the first case, anemia is associated with insufficient intake or loss of iron during bleeding; in the second, the cause of anemia is genetic disorders in the red blood cells themselves, as a result of which they have a shortened life expectancy and are intensively destroyed in the spleen and other organs.

Hypoplastic anemia

But the next type of anemia - hypoplastic, is directly related to primary damage to the bone marrow and disruption of the hematopoietic process. The name of the disease does not accurately reflect its essence, since we are talking not only about anemia, but also about damage to all hematopoietic germs, including those that produce leukocytes and platelets.

Blood tests in such children show a low content of leukocytes, erythrocytes and platelets, and the bone marrow contains a large number of adipose tissue.

Hypoplastic anemia can be congenital or acquired.

Fanconi anemia is a typical representative of the congenital form of the disease. Its peculiarity is that hematological disorders are combined with other congenital malformations, most often of the bones. This may be the presence of additional fingers or the absence of one of them, growth retardation, small head size, irregular dentition, etc. But the hematological component of this symptom complex appears later, at the age of 5-6 years, and then it is this that determines severity and prognosis of the disease.
In addition to the three-lineage lesion, as is the case with Fanconi anemia, there are partial forms, when congenital disorders affect one of the three lines of hematopoiesis, responsible for the production of red blood cells, leukocytes or platelets. These diseases appear already in the neonatal period and are very difficult.

Aplastic anemia

A special group consists of acquired aplastic anemia. In each specific case, it can be very difficult to establish the cause of the disease. It is believed that the disease is directly related to damage to the ancestral stem cell in the bone marrow - the very cell from which hematopoiesis develops in different directions. These may be toxic, infectious or immune effects.

The disease begins acutely, the condition worsens, severe pallor of the skin, bruises, and bleeding of various locations appear. Sometimes the temperature rises.
Acquired aplastic anemia is severe. The disease progresses rapidly and without appropriate treatment can have an unfavorable outcome.

Treatment of aplastic anemia

Treatment of all types of aplastic anemia is difficult task. It is often necessary to carry out a transfusion of blood and its components - red blood cells, thrombus suspension. Comprehensive measures are carried out aimed at restoring hematopoietic function, affecting directly the stem cell.

Who is indicated for bone marrow transplantation and how is it performed?

If there is no effect from such therapy, bone marrow transplantation is indicated for all children with various types of hypoplasia anemia. This procedure is now used quite widely throughout the world for various diseases - hypoplastic anemia, leukemia, various types of immune deficiency, thalassemia, etc. However, it is not indicated for all children with this pathology, but only in those cases where drug therapy turned out to be ineffective.

To perform a bone marrow transplant, a donor is required, who must be compatible with the patient in many respects. This procedure is safe for the donor's health. Compatible donors are usually found among siblings, but in our small families, where there are one or two children, it is difficult to find a donor. Parents, unfortunately, are rarely compatible with their children. Therefore, a new direction is now being developed to use unrelated compatible donors. To search for such donors, there is a special data bank - the International Registry.

The bone marrow transplant procedure itself is not complicated. Bone marrow is removed from the donor under general anesthesia through several injections in the iliac crest. It is passed through special filters and administered intravenously to the patient. But in order to carry out a donor bone marrow transplant, it is necessary to carry out special preparation with drugs that deplete the patient’s own bone marrow. This is the only way to expect the graft to engraft. In this situation, severe complications are possible that can negate the entire procedure. For this purpose, there are special methods for preventing complications and treating them.

Today we can say with confidence that the bone marrow transplant procedure has saved the lives of many patients.

What unites such different diseases?

What unites these different diseases and why is the word “anemia” present in all the names? And the main symptom here is pale skin and a decrease in hemoglobin and red blood cells in a blood test. This is what is defined by the term anemia. But the shades of this pallor and the anemia themselves are different.

For example, with iron deficiency, the shade of pallor is marbled, “blue-white”; with hemolytic anemia, it is lemon yellow; and with aplastic anemia, a gray color of the skin is noted.
Therefore, when examining a patient, the doctor must pay attention to these shades. And this immediately directs the diagnostic thought in the right direction, and the hematological examination allows us to clarify the diagnosis and prescribe correct treatment.
It should be remembered that some types of anemia are more common at a certain age. For example, iron deficiency anemia - in children of the first two years of life and in girls puberty. And if iron deficiency anemia is detected in children of other age groups, then this is most likely associated with hidden bleeding and is defined as chronic hemorrhagic anemia.

The first signs of congenital hypoplastic Fanconi anemia appear at the age of 5-6 years, partial - immediately at birth, and acquired forms can develop at any age. Hereditary hemolytic anemia is usually diagnosed in preschool age, and the earlier, the more severe the disease and the more parents are informed about this disease, that is, if there are already such patients in the family.

It should be noted that with anemia, the decrease in red blood counts can be moderate and very pronounced. Anemia can develop gradually and very quickly. Accordingly, the complaints of patients, their general state will be disrupted to varying degrees.

With moderate anemia, children long time may not make complaints, and only very attentive parents will notice changes in the child’s behavior and condition. With severe anemia and, most importantly, with its rapid development, the child’s condition worsens. He becomes very pale, lethargic, passive, palpitations are felt, and shortness of breath appears. This happens in acute bleeding and hemolytic crises. Iron deficiency anemia, as a rule, develops gradually, and the clinical manifestations of the disease will be more pronounced the later the examination and diagnosis are made.

Blood diseases associated with increased bleeding

Another group of diseases consists of the so-called hemorrhagic diathesis. This also includes diseases of different nature, but they are united by one main clinical sign - increased bleeding.

How and why bleeding stops

Before talking about individual diseases of this group, we need to answer two questions: important issues: why is the blood in intact vessels in a liquid state and does not clot, and how does bleeding stop when a vessel is injured, that is, how is hemostasis accomplished?

This is explained by the fact that the factors involved in the blood coagulation process are in an inactive state in the blood plasma, and, in addition, there are corresponding anti-coagulation mechanisms.

The system begins to work only if an injury to the vascular wall occurs. Then all the necessary mechanisms are included in the process of stopping bleeding - platelets, the vascular wall itself (its ability to contract is important) and the blood coagulation system. Platelets are the first to respond to injury, as they directly line the vessel from the inside. Through complex transformations, they form a hemostatic plug - a “patch” and, together with vascular spasm, provide primary hemostasis.

Upon contact with a damaged vascular wall, special proteins are activated - coagulation factors found in the plasma. The blood clotting process starts, and the primary platelet thrombus is replaced by a dense fibrin clot, which then acts as a hemostatic plug until the injured area of the vessel heals.

Thrombocytopathies

The most common diseases in the group hemorrhagic diathesis- so-called thrombocytopathies. They are based on the qualitative inferiority of platelets when their number is normal.

Because platelets have different properties, there are many different types of thrombocytopathy. All of them are hereditary diseases.

Typically, such patients complain of nosebleeds and a tendency to bruise. Girls may have long and heavy periods.

Most thrombocytopathy is mild, but some rare forms which are severe and require special treatment.

The blood test in such people is normal, but in order to establish what kind of defect there is, it is necessary to conduct complex studies of the dynamic properties of platelets. Often people with this pathology do not consider themselves sick. They stop frequent, light nosebleeds on their own, bruises occur easily but go away quickly, and long-term uterine bleeding considered their specialty. Moreover, there are no special methods for treating thrombocytopathies.

Thrombocytopenic purpura

If increased bleeding is associated with a decrease in the number of platelets (thrombocytopenia), then we speak of thrombocytopenic purpura (TPP). Thrombocytopenic purpura can be an independent disease, or it can be a symptom of other serious diseases. We have already talked about one of them - aplastic anemia.

Thrombocytopenia occurs when an infection, such as a respiratory viral infection, or medication changes the properties of your own platelets. Under these conditions, cells responsible for immunological surveillance perceive them as foreign and begin to produce antibodies against them. And these altered platelets, blocked by antibodies, are captured by special cells of the spleen and destroyed there. If an infection is usually responsible for a change in the properties of platelets, then the resolving factor immediately preceding the development of thrombocytopenia may be repeated infectious diseases, trauma, insolation, hypothermia, preventive vaccinations, etc.

The clinical manifestations of the disease are similar to those described for patients with thrombocytopathies. But, if in the first case they continue constantly, almost throughout life, then in patients with TPP they arise suddenly and are more pronounced.

The main elements of the rash are bruises and petechiae.

Bruises are blue spots located everywhere, but mainly on the lower legs. Bruises arise from minor injuries and have different shapes and sizes. Depending on how long ago they appeared, they have different colour- from blue to yellow-brown.

Petechiae are pinpoint rashes that are also most often located on the legs. The number of such hemorrhages ranges from single to very abundant. At severe forms hemorrhages in the mucous membranes and bleeding are observed. Bleeding from the gums and nasal mucosa is especially common. Sometimes the bleeding is so severe that it is necessary to pack the nasal passages and transfuse freshly prepared platelet mass. TPP is especially dangerous for girls at puberty. They may experience heavy uterine bleeding.

Newly diagnosed TPP progresses as follows: acute illness, which can end in recovery or become the beginning of a serious, chronic illness.

Treatment is aimed at destroying aggressive antiplatelet antibodies and eliminating bleeding. For this purpose it is prescribed special drugs, and in case of heavy bleeding it is even necessary to transfuse blood products. For most children this is enough. The platelet count is restored, hemorrhages and bleeding stop.

However, this does not mean recovery for all patients. If the disease has become chronic, then episodes of bleeding are repeated, and then the issue of radical treatment is decided - removal of the spleen. Just as with hemolytic anemia, the organ where cell destruction occurs is removed. Removal of the spleen (splenectomy) also gives good results and is the method of choice for severe chronic forms.

If at the beginning of the disease it is difficult to talk about any preventive measures, then with an existing disease it is possible and necessary to prevent exacerbations. To do this, it is necessary to cure all foci of chronic infection - carious teeth, inflammation of the adenoids, chronic tonsillitis and others. Children are exempt from physical education during this period. It is not recommended to sunbathe or take drugs that interfere with the dynamic properties of platelets - primarily aspirin, chimes, indomethacin, etc. A medical exemption from preventive vaccinations is given. Children should be followed up by a hematologist for five years after TBI.

Hemorrhagic vasculitis

Another disease that should be discussed and which is also quite common in children is the so-called hemorrhagic vasculitis. With this disease, antibodies are also produced, but they are directed not against blood cells, but against the cells lining the inside vascular wall. These cells are called endothelium.
Antibodies destroy the endothelium and make the vascular wall easily permeable in both directions - from tissues to blood and from blood to tissues. Substances enter the blood from the tissues that further destroy the vascular wall, and blood sweats from the vessels into the tissue, which causes a rash on the skin. But this rash differs from bruises both in nature and location. It appears as pink round spots (papules) on the legs, arms, and buttocks. But there is no rash on the face or torso.

Hemorrhagic vasculitis is also characterized by pain and swelling of the small joints of the arms and legs, and abdominal pain. Sometimes there may be blood in the urine. As with TPP, the disease is preceded by some kind of infection, and then, with the action of a resolving factor, hemorrhagic vasculitis develops. The disease can be mild or severe, and has a tendency to early exacerbations.
Treatment of hemorrhagic vasculitis has its own characteristics. Since we are talking about vascular damage, it is necessary to take measures aimed at preventing intravascular coagulation. For this purpose, a special drug, heparin, and medications that prevent platelets from sticking together are prescribed - the so-called disaggregants.
It is also necessary to treat (sanitize) all foci of chronic infection. Treatment is usually effective. Children recover, but the duration of treatment varies depending on the severity of the disease - from one to several months.

Hemophilia

Hemophilia occupies a special place in the group of hemorrhagic diathesis. The disease is based on a congenital, hereditary disorder of blood clotting due to a lack of factors VIII or IX. Accordingly, hemophilia can be A or B. The peculiarity of inheritance in hemophilia is that the disease is gender-linked. The disease is transmitted from grandfather to grandson through his daughter, who is called the “conductor.” Such a woman has a high probability of giving birth to hemophilic sons, and in such families there are almost always also men suffering from hemophilia.

Unlike TPP, hemophilia is characterized by prolonged, difficult to stop bleeding, hemorrhages in the joints, subcutaneous and large intramuscular hemorrhages - hematomas, which then take a long time to resolve.

The first signs of the disease may appear already in the first year of life, when teeth erupt or when the child begins to walk, often hurts himself and falls. However, already in the neonatal period there may be prolonged bleeding from the umbilical wound or extensive subperiosteal hemorrhage on the head, the so-called cephalohematoma.

Repeated hemorrhages in the same joint lead to its deformation, and pain in the joint limits movement. This causes muscle atrophy, causing children to stop walking. The leg is fixed in a bent position with a “hook”. The disease seems to “shackle” the child. This happens if it is not possible to provide timely and correct treatment to patients.

And the treatment of hemophilia is a complex problem, since it requires the presence of a powerful blood transfusion station, where special preparations containing concentrates of factors VIII and IX are prepared. In addition, patients must constantly engage in physical therapy; swimming in the pool is recommended. From physical education classes at school and big physical activity children are released. Timely sanitation of the oral cavity is necessary, since severe bleeding occurs during tooth extraction. If all necessary treatment and preventive measures are carried out, patients can manage sufficiently active image life.

Acute leukemia in children

Now let's talk about acute leukemia. This disease, which is oncological in nature, is classified in the group of oncohematological ones, since we are talking about damage to the main organ of the blood system - the bone marrow.

The disease is based on a disruption (mutation) of one of the ancestral stem cells of the bone marrow, which gives rise to a new diseased generation (clone) of cells. These cells escape the control of regulatory mechanisms and begin to multiply uncontrollably, losing the ability to mature. As a result, a huge number of such diseased cells - blasts - accumulate in the bone marrow.

As blast cells leave the bone marrow, they are carried throughout the body by the bloodstream and enter various organs and tissues. They find especially favorable conditions in the liver, spleen and lymph nodes with leukemia. It was in these organs that the function of hematopoiesis was carried out in the early stages of intrauterine development. Here these diseased cells multiply unhindered and cause organ enlargement.

And in the bone marrow another dramatic situation continues. Having accumulated in a huge number, these immature cells displace normal hematopoietic tissue, resulting in only a small amount of mature, functional full cells- erythrocytes, leukocytes, platelets. With their deficiency, as we have already discussed above, the skin becomes pale, bruises appear and there is a tendency to infectious diseases. And the appearance in a blood test of young, immature cells - blasts, which are not able to perform the duties of healthy, mature blood cells, is the main sign of leukemia.

Thus, doctors today have a fairly good idea of how this disease develops and can explain certain symptoms, but still no one knows the exact cause of leukemia.

Scientists suggest that so-called proto-oncogenes pre-exist in the body, which are in an inactive state and have no pathological significance for the body. But under the influence of some, often unknown to us, external influences they are activated, embedded in healthy cell and cause its change (mutation). And she is already creating a clone of similar cells - diseased cells. This proto-oncogene is similar to a viral DNA particle, which is why this theory of leukemia is sometimes called a viral theory. However, it has nothing to do with the spread of ordinary viral diseases, such as influenza, measles, rubella, etc. Leukemia is a non-contagious disease, and you cannot become infected with it through contact with a sick person.

Leukemia - and not hereditary disease. Sick children are usually born in healthy families, in which cancer patients are no more common than among other population groups. Repeated cases Leukemia in children in the same family is extremely rare, although it does occur in identical twins. A predisposition to leukemia is observed in children with Down syndrome, as well as in other diseases that are based on genetic disorders.

Parents who had leukemia in childhood give birth to healthy children. At the same time, there are cases of congenital leukemia, when the disease manifests itself in a child already in the first three months of life. Moreover, the parents of such children are practically healthy people. This happens if one of the parents has a mutation directly in the germ cells, without affecting the entire body. Then pathological genetic information is transmitted to the child, and children can be born with any congenital disorders, including leukemia.

This is what bone marrow looks like in leukemia

A few words about the mutations themselves. In the body, mutations, that is, cell changes, occur constantly, at approximately the rate of one cell per hour. But in a healthy body there is a powerful immunological defense that perceives these cells as foreign and destroys them.
Thus, the development of leukemia, like other oncological diseases, is possible only with an unfavorable combination of the action of mutagenic factors and a weakening of the body’s defenses, including anti-cancer immunity.

We know even less about the mutagenic factors themselves that bring these proto-oncogenes out of their dormant state. The role of ionizing radiation is described, as convincingly demonstrated by the consequences of the explosion atomic bombs in Japan. In the cities of Hiroshima and Nagasaki, leukemia occurs several times more often than in other cities.

However, for today's leukemias, for example, in Russia, this does not matter. There is no increase in the frequency of leukemia and other cancers in children after the Chernobyl accident. Rather, we are talking about the damaging effects of small doses of radiation. Describes the role of some chemical substances, occupational hazards among parents and other factors. However, in each specific case it is rarely possible to establish the cause of the disease.

You should also dwell on the differences between childhood and adult leukemia. In children there are mainly sharp forms, originating from immature lymphocytes. Approximately 80% of childhood leukemias are acute lymphoblastic leukemia (ALL), and only 15-20% are acute myeloid leukemia (AML). Chronic forms Leukemia in children is extremely rare. For adults, the picture is the opposite. They are more likely to have chronic leukemia, and of acute leukemia, AML is more common and ALL is less common. These differences are associated with the anatomical and physiological characteristics of the child’s body.

Both very young children and very old people suffer from leukemia. The peak of childhood leukemia occurs in preschool age. Less commonly, leukemia occurs in children under one year and after 10 years.

The first signs and course of leukemia in children

The disease develops gradually. From the onset of the disease to the appearance of the first clinical signs of the disease, at least 1.5-2 months pass. During this time, pathological, “sick” cells accumulate, and quantity turns into quality - the disease makes itself felt.

But even during these 1.5-2 months, complaints already appear, which parents do not always pay attention to. This is primarily a change in the child’s behavior. He gets tired, refuses to eat, studies worse, loses interest in peers and games. Due to a decrease in the body's defenses already in initial period leukemia, colds and other infectious diseases that occur with an increase in temperature are possible. Sometimes an increase in temperature can be directly related to the development of leukemia itself. If during this period you consult a doctor and do a blood test, you can already notice some changes, which are not yet sufficient to make a diagnosis, but which already make you think and continue to monitor the sick child.

Then more definite signs of the disease appear.

Some children complain of pain in their legs and spine. The pain is persistent and appears in one area or another. Such patients often stop walking and are treated for a long time by traumatologists and cardiologists for “trauma” or polyarthritis.

The appearance of increasing pallor of the skin, bruises, an increase in the size of the liver, spleen and lymph nodes makes the suspicion of leukemia more likely.

And in the blood test at this time there are already all the changes characteristic of leukemia: a decrease in the content of hemoglobin, red blood cells, platelets, a sharply accelerated ESR. But the number of leukocytes can be low, high, or very high. The more blast cells from the bone marrow into the blood, the higher the number of leukocytes. The appearance of blasts in a blood test, which never occur in a normal analysis, is absolute sign acute leukemia.

In this situation, a bone marrow puncture is required. This is necessary not only to confirm that the bone marrow is entirely represented by blast cells. It is important to study the structural features of these cells, whether they belong to the T- or B-cell line, and to identify the presence of cytogenetic disorders. Obtained in this way additional information help diagnose various types of leukemia, identify additional risk factors for unfavorable course of the disease and prescribe adequate treatment.

And only after this is it possible to have a substantive conversation with parents about the diagnosis, treatment and prognosis of the disease.

Modern methods of treating leukemia in children

Great strides have been made in the treatment of childhood leukemia over the past 10-15 years. If previously the diagnosis sounded like a death sentence, now most children recover and fully adapt to a full life. Today it is a disease that can and should be treated.

Since the early 90s, new treatment programs have been used in the treatment of leukemia in children. They use several drugs with antitumor effects simultaneously, and these drugs are prescribed in large doses, much higher than usual therapeutic ones. The course of treatment is designed for a long period, which is a total of 2 years.
Treatment programs are developed taking into account the type of leukemia and the presence or absence additional factors the risk of possible relapse (return) of the disease. That is why it is so important, when making a diagnosis, to carefully conduct a bone marrow examination and select an appropriate treatment plan (protocol). Therapy is usually accompanied by severe infectious complications and increased bleeding. However, there are methods for preventing and treating these complications, which allows most children to completely complete the intended treatment program.

In particular severe cases, When drug therapy does not guarantee success, the question of the need for a bone marrow transplant is raised. To do this, the patient’s relatives are examined and, if they are suitable, they are used as donors. We talked in more detail about the bone marrow transplant procedure in the section on hypoplastic anemia.

In many ways, the results of treatment for leukemia depend on the parents, on how promptly they consulted a doctor and how selflessly they fought for the child’s life, helping medical personnel during treatment.

OBSERVATION BY A HEMATOLOGIST

All children with an established hematological diagnosis are under the supervision of a hematologist. This is a dispensary group of patients.

Some continue the treatment started in the hospital, others are observed after discontinuation of therapy, and still others undergo the entire process of diagnosis, treatment and follow-up on an outpatient basis.

Children are usually invited for control once a week, unless the doctor individually prescribes otherwise. The child is examined by a doctor and taken necessary tests, corrections are made in treatment.

Some children are treated in a day hospital or, as it is also called, a “day hospital”. Here the child receives all the necessary diagnostic and treatment procedures during the day, after which he goes home. Treatment in this mode can last for quite a long time. Such day hospital its capabilities are no different from a specialized hematology department. Here you can do Ultrasound, X-ray, CT, NMR and others diagnostic procedures, perform the necessary puncture studies, blood transfusion, administer medications, consult with specialists of any profile.

World experience in managing patients with a variety of diseases has shown that this practice is optimal for both the child and the family, if the condition of the sick child allows it.
Even for patients with leukemia, treatment programs are designed in such a way that they can spend most of their time at home in a familiar, family environment. And this not only has a beneficial effect on the child’s mood and the situation in the family, but is also an important factor in the prevention of nosocomial infections.

Many children with blood diseases are disabled from childhood. Disability is established for 5 years or immediately up to 16 years, depending on the disease. During all this time, the hematologist decides not only purely medical issues, but also a whole range of social problems that families of sick children face: homeschooling, provision of additional days off, admission to physical education, preventive vaccinations, and referral to sanatorium treatment.

Issues of career guidance are discussed with older children. For example, patients with hemophilia are not recommended to choose specialties associated with the risk of increased injury. At the same time, adolescents who suffered from leukemia in early childhood are considered practically healthy people and can choose a specialty in accordance with their wishes.

Nowadays the question is often discussed: up to what age should a pediatrician and, in particular, an oncologist and hematologist observe their patients? Today, such children are under supervision until they are 15 years old. But that's it larger number Experts believe that these terms should be increased to 18 years, and such a law has already been adopted.

In some countries, patients who have childhood-onset or childhood-onset adolescence chronic diseases, are observed by pediatricians until the age of 21.
One thing is undeniable: adolescents with oncological and oncological-hematological pathologies should be managed according to treatment programs adopted in pediatric practice.

Today, pediatric hematology and especially pediatric onco-hematology are developing successfully. Last years were marked by a significant enrichment of new knowledge about blood diseases.

IN daily practice methods have been introduced for the immunological determination of tumor and leukemia variants, cytogenetic and molecular biological studies, ultrasound, CT and NMR methods, new diagnostic methods infectious complications and others.

Significant progress has been made in the treatment of patients with leukemia and other blood diseases. This became possible thanks to the establishment of close scientific and practical ties with foreign colleagues, the introduction of new modern methods examination and treatment of patients.
Pediatric hematology as a separate branch of modern hematology attracts special attention from researchers. Due to the age-related variability of the child’s body and, in particular, the blood system, diseases in children proceed differently from adults and in some cases have a better prognosis. Some diseases that are common in adults are not found in children at all, and vice versa.

On the other hand, it is in childhood that hereditary blood diseases and the consequences of the damaging effects of various factors during the intrauterine formation of the blood system or immediately after birth are often detected. And the quality of a person’s life in the future depends on how pediatric hematologists cope with them.

The near future gives particular optimism complete cure leukemia and other tumors in children, which we have come close to today.

Who is a Hematologist:

Hematologist (Greek haima, haimatos blood + logos teaching) is a medical specialist (therapist) trained in diseases of the blood and hematopoietic organs, engaged in therapeutic and preventive or research activities in this area.

What is the competence of a Hematologist:

Competent in areas of medicine that studies the structure and functions of the blood system (the blood itself, hematopoietic and hematopoietic organs), the causes and mechanisms of development of blood diseases and develops methods for their recognition, treatment and prevention.

What diseases does a Hematologist treat?

Iron deficiency anemia

The most common diseases of the blood system are anemia. We are talking about anemia when red blood levels - hemoglobin and red blood cells - decrease. The concept of anemia includes diseases of different nature, but most often they are associated with a lack of iron and are therefore called iron deficiency anemia.

After birth, especially in the first year of life, the child grows rapidly. Over the course of a year, his weight triples, and his blood volume increases 2.5 times.

Accordingly, the need for iron increases sharply. And this is where the biggest difficulties arise.
Iron enters the body only from the outside - with food.

Different foods contain different amounts of iron, and, unfortunately, milk contains the least amount of it. And if we remember that milk is the main food in the first year of life, it becomes clear why children of this particular age group so often suffer from iron deficiency anemia.

Properly replenished iron deficiency leads to the child's recovery from anemia.

There is another group of children most susceptible to iron deficiency anemia - girls of puberty (puberty). At this time, a rapid restructuring of the body also occurs and the need for iron increases sharply. The diagnosis in these patients is usually made late. The disease accumulates over a long period of time and manages to develop the whole complex of symptoms characteristic of chronic sideropenia (the so-called iron deficiency). These are primarily fatigue, drowsiness, sudden deterioration and perversion of appetite. Patients cannot stand the taste and smell of meat, fish and chew chalk and cereals with pleasure. Their skin is dry, their hair and nails are dull and brittle.

Significant changes are noted in the mucous membranes of the mouth and pharynx, including disruption of the act of swallowing.

In addition, menstruation begins at this age, and in girls with any platelet disorders, they can take the form of prolonged, heavy bleeding. In this situation, iron deficiency anemia also develops due to loss of iron.

Iron deficiency anemia also occurs with prolonged bleeding in small portions that are not visible to the naked eye. Such blood loss most often occurs from the mucous membrane of the gastrointestinal tract in various diseases, such as hiatal hernia, polyps, vascular tumors (hemangiomas), duodenal ulcers, etc.

A necessary condition for the recovery of patients in whom iron deficiency has developed as a result of chronic blood loss is treatment of the underlying disease that caused the bleeding, and often through surgery. Only after these measures is it possible to restore iron levels and recover from anemia.

Hemolytic anemia
The second most common group of anemias is the so-called hemolytic anemia. In these diseases, the bone marrow produces a sufficient number of red blood cells, but for various reasons they have a shortened life span and are quickly destroyed.

Much less common are the so-called espherocytic hemolytic anemias, which are inherited in a recessive manner and are severe.

In this case, the cause of accelerated cell destruction is a congenital deficiency of one of the many enzymes contained in red blood cells.

As a result of the described disorders, red blood cells in hemolytic anemia, as we said above, have a shortened life expectancy.

With Minkowski-Choffard anemia, they are destroyed in the spleen, that is, in the organ where old, spent cells are destroyed under normal conditions.

In non-spherocytic hemolytic anemias, hemolysis (cell destruction) occurs wherever there are macrophages that can capture altered red blood cells, for example, in the liver, bone marrow and other organs.
The main clinical signs for all types of hemolytic anemia are pale skin, jaundice and an enlarged spleen. Such patients are constantly moderately pale and yellow, but periodically their condition worsens sharply, the temperature rises, and the pallor and yellowness of the skin increases. These are signs of exacerbation of the disease, so-called hemolytic crises. During this period, patients need special treatment measures, and often blood transfusions.

There is also a surgical method for treating Minkowski-Choffard anemia. This is the removal of the spleen - splenectomy, that is, removal of the organ responsible for the accelerated destruction of red blood cells.
Even if Minkowski-Choffar anemia is mild and crises are rare, over time such a serious complication as cholelithiasis develops. Removal of the spleen leads to recovery in all patients. Blood tests normalize, jaundice disappears, and the formation of gallstones stops. However, the genetic defect remains, which is inherited. And even parents who have undergone surgery have a high probability of giving birth to a child with hemolytic anemia. But they already know about the miraculous effects of splenectomy.
This cannot be said about non-spherocytic hemolytic anemias. Fortunately, they are rare. Since the destruction of red blood cells occurs in many organs, removal of the spleen gives a partial effect or no effect at all.

These are anemias, the origin of which is not directly related to dysfunction of the bone marrow.

Hypoplastic anemia
But the next type of anemia, hypoplastic, is directly related to primary damage to the bone marrow and disruption of the hematopoietic process. The name of the disease does not accurately reflect its essence, since we are talking not only about anemia, but also about damage to all hematopoietic germs, including those that produce leukocytes and platelets.
In blood tests in such children, the content of leukocytes, red blood cells and platelets is low, and the bone marrow contains a large amount of adipose tissue instead of normal hematopoietic tissue.

Hypoplastic anemia can be congenital or acquired.
Fanconi anemia is a typical representative of the congenital form of the disease. Its peculiarity is that hematological disorders are combined with other congenital malformations, most often of the bones. This may be the presence of additional fingers or the absence of one of them, growth retardation, small head size, irregular dentition, etc. But the hematological component of this symptom complex appears later, at the age of 5-6 years, and then it is this that determines severity and prognosis of the disease.

In addition to the three-lineage lesion, as is the case with Fanconi anemia, there are partial forms, when congenital disorders affect one of the three lines of hematopoiesis, responsible for the production of red blood cells, leukocytes or platelets. These diseases appear already in the neonatal period and are very difficult.

Aplastic anemia

A special group consists of acquired aplastic anemia. In each specific case, it can be very difficult to establish the cause of the disease.

It is believed that the disease is directly related to damage to the ancestral stem cell in the bone marrow - the very cell from which hematopoiesis develops in different directions. These may be toxic, infectious or immune effects.

What organs does the Hematologist deal with:

When to contact a Hematologist:

When and what tests should be done:

Diagnosis of anemia:

Complete blood count with reticulocytes;
- Transferrin saturation percentage;
- Ferritin;
- Iron-binding ability.

What are the main types of diagnostics usually performed by a Hematologist:

Today, methods of ultrasound diagnostics (US), computed tomography (CT), and nuclear magnetic resonance (NMR) are also successfully used in the diagnosis of blood diseases. These modern studies make it possible to determine the size and structure of organs, various bone disorders, and identify tumor formations of various localizations.

The diagnosis of leukemia can be a real shock for a person and especially for parents.

The following tips will help you cope with the situation:

Learn all the information available about the type of leukemia you have been diagnosed with and its treatment. This information will help you make the best choice and you will know what to expect.

Do not give up. Healthy diet, adequate rest and regular physical exercise will improve the general condition of the body.

Connect with other people or families who are dealing with this condition. Ask your doctor about support groups in your area. You can also find people online who share this problem with you.

American scientists from the Mayo Clinic in Minnesota discovered that the antioxidants contained in green tea kill cancer cells and reduce the symptoms of cancer, writes Telegraph. Experts have been researching the anti-cancer properties of green tea since the 1970s and have found that in countries where people drink green tea, lower incidence of cancer.
In 2004, a study on mice showed that the antioxidants contained in green tea can kill leukemia cancer cells. Preliminary results also suggest that the tea may have the same effect on patients suffering from chronic lymphocytic leukemia (one of the most common forms of leukemia).

For patients with this disease, doctors prescribed green tea extract - 400-2000 mg 2 times a day. Most of them noted a reduction in lymph nodes by about 50% or even more. According to researchers, green tea can stabilize the condition of patients with early stage and slow down the progression of the disease.

Hematologist is a medical specialist (therapist) who knows the issues of hematopoietic organs and blood and is engaged in research or therapeutic and preventive activities in this field of medicine.

Competence of a hematologist

A doctor in this area of medicine studies the structure and functions of the blood system, the causes and mechanisms of development of blood-related diseases, and develops methods for their recognition, treatment and prevention. A hematologist studies:

Pathogenesis and etiology of the hematopoietic system.
Develops and implements innovative methods for diagnosing diseases of the hematopoietic system and bone marrow.
Study the clinical signs of blood pathology.
Treats and prevents blood pathologies.

What a qualified hematologist must know

A hematologist must have the following knowledge:

Physiology, morphology, embryogenesis of the hematopoietic system and blood cells.
Characteristics and properties of plasma and serum.
Properties that the hematopoietic system exhibits in pathological and non-hematological blood diseases.
Fundamentals of immunohematology, hemostasiology and hematology.
Knowledge of the medical examination system and methods of diagnosing blood and bone marrow.
Methods of chemotherapy.
Fundamentals of medical ethics, human morality (deontology).

Which doctors do hematologists work closely with?

Hematologists collaborate with doctors in the following areas of medicine:

Oncologist.
Surgeon.
Gynecologist.

What organs are within the competence of a hematologist?

The list of organs includes the spleen, blood and bone marrow.

What diseases does a hematologist treat?

According to statistics, 9% of all human diseases are related to blood. A hematologist determines leukemia and anemia. The most common diseases are:

Blood diseases, including anemia, which is divided into:
- hemolytic, aplastic;
- iron deficiency, Mediterranean (thalassemia);
- folate deficiency, posthemorrhagic;
- against the background of chronic pathology, B12 deficiency.
Leukemia includes:
- chronic and acute.
Diseases:
- Guglielmo's disease;
- hemoblastosis;
- lymphosarcoma;
- lymphogranulomatosis;
- increased bleeding;
- thrombocytopenia;
- hemophilia.

Symptoms of the main diseases treated by a hematologist

Iron deficiency anemia. Anemia - a decrease in red blood counts (red blood cells and hemoglobin). The disease is often associated with a lack of iron, hence the name (iron deficiency). With its deficiency in the body, metabolic processes are disrupted. In children, anemia of this type most often develops before the age of 2 years. The reasons may be conditions of intrauterine development (severe toxicosis of a pregnant woman or chronic diseases, as well as a vegetarian diet). In the first year of life, with intensive growth, the baby must receive iron from food, but there is not enough of it in the milk he eats.

When observed by a pediatrician, external signs, iron deficiency can be determined, so it is important to introduce complementary foods, juices, and mixtures enriched with vitamins in a timely manner. A special place in the group of children is given to girls of puberty (maturation period). At this time, a rapid restructuring of the body occurs, so there is a need for iron. The diagnosis in this case is made late, since the disease accumulates gradually and over a long period of time. Signs: fatigue, loss of appetite, drowsiness. At the same time, patients enjoy eating chalk and cereals and cannot stand the smell of meat and fish. Hair is dull, skin is dry, nails are brittle. From the mucous membranes of the oral cavity, disturbances in the act of swallowing can be noticed. Girls with iron deficiency experience menstrual irregularities (prolonged with heavy bleeding).

Iron deficiency anemia can occur due to prolonged bleeding in small portions in diseases such as hiatal hernia, polyps, hemangiomas (vascular tumors), duodenal ulcers, etc. In case of chronic blood loss due to which iron deficiency has developed, it is necessary to treat the underlying disease.

Hemolytic anemia. This is when the bone marrow produces a sufficient number of red blood cells, but due to a number of reasons they have a shortened life span, and therefore are destroyed quickly. This disease is either hereditary or congenital. Among hereditary anemias Microspherocytic hemolytic anemia is common (a genetic defect located in the erythrocyte membrane). Hierospherocytic hemolytic anemia is rare (it is inherited in a recessive manner and is severe).

Non-spherocytic hemolytic anemia (cell destruction). It occurs everywhere where macrophages are present that detect changes in red blood cells (in the liver, bone marrow, etc.). Clinical signs: enlarged spleen, pale skin, jaundice. During an exacerbation (hemolytic crisis), patients need special treatment measures, often with blood transfusions. The surgical treatment for Minkowski-Choffard anemia is the removal of the spleen, which is responsible for the destruction of red blood cells. This operation leads to the recovery of patients (blood normalizes, the formation of gallstones stops, jaundice disappears). But the genetic defect is inherited.

Hypoplastic anemia is associated with primary damage to the bone marrow and impaired hematopoiesis. Hematopoietic sprouts that produce platelets and leukocytes are affected. In such children, the blood test shows a low content of leukocytes, red blood cells and platelets, and the bone marrow contains a lot of fatty tissue instead of hematopoietic tissue. This type of anemia can be acquired or congenital. The congenital form of the disease is Fanconi anemia. The disease is difficult.

Aplastic anemia - acquired. It is difficult to establish the cause of the disease. The disease is associated with damage to the ancestral stem cell in the bone marrow. These can be immune, infectious and toxic effects. The disease is acute (pallor of the skin, bleeding of various locations, bruises, fever). It is severe and progressive, so without treatment there may be an unfavorable outcome.

When should you contact a hematologist?

The presence of hematological disorders has a number of significant signs, including:

Weakness, weight loss.
Increase in temperature for no apparent reason.
Excessive pallor of the skin, loss of appetite.
Excessive sweating, redness of the facial skin.
Tingling of fingertips and numbness.
Reduced rate hemoglobin.
The appearance of tumors under the armpits, in the groin or neck and their enlargement.
Bruises appear for no apparent reason.
After water procedures, skin itching.
Long-term healing of scratches, cuts and bleeding wounds.
Violation menstrual cycle chronic form.
Headache without obvious cause.

If your place of residence has an increased background radiation, if you are planning a pregnancy, you should undergo examination by a hematologist in order to protect yourself from unpleasant surprises associated with disruption of the hematopoietic system.

What to do before visiting a hematologist

Before visiting a hematologist, you must adhere to certain conditions:

Do not eat for 12 hours.
Do not drink alcohol or smoke during the day.
Not to accept medications, and if you can’t not take it, be sure to notify your doctor about exactly what medications you took.
The day before visiting the doctor, reduce your fluid intake.

These conditions must be met in order to receive accurate analyzes, punctures and other manipulations.

Laboratory tests and diagnostics that a hematologist may prescribe

Complete blood count with reticulocytes.
Study of transferrin saturation percentage.
Ferritin.
Study of iron-binding ability.

Diagnostics:

Biopsy of lymph nodes and abdominal cavity.
Ultrasonography areas of lymph nodes with histological examination.
X-ray of blood of a certain specificity.
Checking blood clotting (coagulogram).
Bone marrow examination (morphological) with sternal puncture.
Computer tomogram (CT).
Scintigraphy for bone scanning.
NMR (nuclear magnetic resonance).

The competence of a hematologist is oncohematology, which deals with malignant pathologies of the hematopoietic system (myeloid leukemia and leukemia).

If you are diagnosed with leukemia, study all the available information that will help you come to a conclusion. the right choice and knowledge of what to expect with this disease.

Diet, rest and regular exercise improve the condition of this disease.

Communication with people who have the same problem helps you cope and survive mentally and physically. Ask your doctor about support groups in your area, or connect online with people who know this problem who can share it with you.

Drink green tea. The antioxidants it contains kill cancer cells and reduce symptoms of the disease. When consumed of this drink, a decrease in lymph nodes of 50% or more was noticed.

Only a highly qualified hematologist can establish the causes of blood disease and timely notice changes in its composition, as well as prescribe correct and effective treatment.

Hematologist- a specialist in the field of hematology, studying the hematopoietic organs, the blood itself and its components, as well as diseases circulatory system.

Hematological diseases are considered systemic. In practice, this means that they are very difficult to diagnose, and sometimes the patient is referred from one specialist to another until it is clarified the real reason feeling unwell.

To understand what diseases a hematologist treats, it is important to understand the functions of the circulatory system and the factors contributing to the development of pathologies in this area. So, blood is mainly responsible for:

transportation of oxygen and other important substances to organs and tissues;
removal of carbon dioxide and some waste products from organs and tissues;

Thus, the coordinated functioning of the whole organism depends on the normal functioning of the circulatory system.

Factors that provoke blood diseases

bleeding;
deficiency of iron, folic acid or vitamin B12;
bone marrow diseases;
intestinal pathologies;
hereditary diseases;
injuries;
surgical intervention;
During pregnancy and breastfeeding.

Diseases treated by a hematologist

According to statistics, about 8% of all human diseases are associated with pathologies of the circulatory system and hematopoietic organs. Hematology as a science can be divided into sections, depending on the nature of the disease. Here are some of them:

General hematology

anemia (including in pregnant women) - a condition in which the level of hemoglobin and red blood cells is reduced;
hemophilia is a hereditary disease characterized by poor clotting, bleeding begins even from a small cut;
coagulopathy;
increased bleeding;
idiopathic thrombocytopenic purpura.

Oncohematology

lymphomas;
lymphocytic leukemia;
myeloid leukemia;
multiple myeloma;
Waldenström's macroglobulinemia.

When should I go for an appointment?

You should make an appointment with your doctor if you have been experiencing the following symptoms for some time:

weakness, constant or intermittent;
persistent and prolonged increase in temperature, subfebrile or up to 38 O C for unknown reasons;
pale skin;
abdominal pain of unknown etiology;
causeless pain in joints, spine, bones;
poor appetite;
night sweats;
enlarged lymph nodes;
bruises on the skin that did not appear as a result of injury or bruise;
prolonged bleeding from cuts, frequent nosebleeds;
regular irregularities in the menstrual cycle, accompanied by heavy bleeding;
enlarged spleen;
if in general analysis blood tests, atypical changes were detected in one of the blood components.

Pregnancy and hematology

It is worth mentioning that it is important for a woman planning a pregnancy or those who are already expecting a baby to be examined by a hematologist. Most often, a doctor treats anemia in a pregnant woman. It is dangerous because it leads to fetal hypoxia. Therefore, if tests show a low hemoglobin level, appropriate treatment is prescribed.

During pregnancy, blood elements undergo qualitative and quantitative changes which need to be monitored periodically. Childbirth itself is a process accompanied by blood loss, so expectant mothers' blood must be checked for clotting ability.

Children and hematology

Diseases of the circulatory system, unfortunately, occur not only in adults, but also in children. They can be diagnosed in a child in infancy. Their course can be affected by pathologies of intrauterine development, non-compliance with the rules of artificial feeding, genetic predisposition, some infectious diseases.

The earlier the disease is recognized, the more effective the treatment will be. Children are treated by a pediatric hematologist, although the range of diseases is almost the same as in adult patients.

At the doctor

The appointment with a hematologist is as follows. First, the doctor conducts an external examination, in particular, of the lymph nodes in the neck, armpits, and groin area, and collects an anamnesis. That is, he asks the patient about troubling symptoms, when and under what circumstances they appeared, and what diseases had already been ruled out before coming to the hematologist’s office. Based on the collected data, the specialist at the reception will decide which of the tests or instrumental studies will need to be done.

The following procedures may be prescribed:

abdominal organs and peripheral lymph nodes;
general blood analysis;
a complete blood test (determines the leukocyte formula, platelets, reticulocytes, etc.);
coagulogram, which is a test for clotting;
myelogram - puncture and morphological examination of the bone marrow;
biopsy of lymph nodes together with histology study;
immunophenotyping;
computed tomogram to examine internal organs;
scintigraphy (bone scan);
genetic research;
x-ray blood test.

Some tests can be taken on the day of the visit to the doctor, so it is recommended not to consume food 10-12 hours before the visit (tests are taken on an empty stomach), alcohol and tobacco products. If the patient is taking medications, be sure to tell the doctor about it. If possible, they do not drink on the day of the study. Also, liquid intake is limited during the day.