Thrombocytopenic purpura: risk factors, types, development, diagnosis, therapy, prognosis. Hemorrhagic diathesis. hemophilia. thrombocytopenic purpura. hemorrhagic vasculitis. hemorrhagic telangiectasia

Hemorrhagic pathologies are specific diseases blood. In children's practice they occur quite often, they differ quite severe course. Thrombocytopenic purpura occurs quite often in children.

Etiology

Currently, there are many hemorrhagic pathologies. Their development is led by the most different reasons. One such disease is thrombocytopenic purpura. This pathology tops the list of hemorrhagic diseases in both children and adults. If you focus on statistics, it can be noted that it occurs in 40-70% of patients with various pathologies blood.

This pathology is characterized strong decline in the peripheral bloodstream the total number of platelet cells. This is due to the presence of an immune conflict between blood platelets and antigens. It should be noted that the anatomical parameters of the spleen remain normal. Doctors note that more and more cases of this hemorrhagic disease are registered every year.

Babies suffer from this pathology much more often than boys.

Single reason The occurrence of the disease has not yet been established. For a long time, scientists could not understand why this blood pathology occurs in babies. Only modern developments and scientific research has helped experts get some answers to their questions.

The development of thrombocytopenic purpura is caused by exposure to a variety of factors. The most common and scientifically based include:

Long-term use of certain types of medications. Scientists have proven that more than 60 different medications can cause symptoms of this hemorrhagic disease to appear on the skin of a child. Such drugs include quinine, quinidine, sulfonamides, heparin, furosemide, dipyridamole, digoxin, metabolites of acetylsalicylic acid, paracetamol, some types of beta blockers, thiazides, cephalosporins, ampicillin, levamisole and many others. Predicting the development of hemorrhagic disease in a particular child is an almost impossible task.

Genetic defects. Many European scientists believe that heredity plays a very important role in the development of the idiopathic variant of the disease. Numerous scientific experiments are now being conducted that should prove or disprove this theory. Existing genetic disorders can lead to the development of autoimmune inflammation and the appearance of adverse symptoms of hemorrhagic disease.
Consequences of past infectious pathologies. Toxic effects of waste products pathogens on the children's body leads to the development of various inflammatory immunological reactions. In children with congenital immunodeficiency conditions, the risk of thrombocytopenic purpura is increased several times.

Intrauterine infection. The first signs of hemorrhagic disease form in the baby while still in the womb. A woman suffering from thrombocytopenic purpura can transmit a number of autoimmune antibodies to her child through the placental bloodstream. This clinical situation leads to the fact that unfavorable symptoms of the disease appear in a newborn baby already in the first months of life.

Pathogenesis

For a long time, scientists could not establish what the mechanism of development of thrombocytopenic purpura in children is. For many decades, various theories have been maintained that explain the pathogenesis of this hemorrhagic disease. IN Lately(due to the advent of new laboratory equipment and improved diagnostic methods), new knowledge is emerging about the mechanism of occurrence of thrombocytopenic purpura in children.

During pathological process V children's body a large number of specific protein molecules appear. These are called antiplatelet antibodies. These substances begin to interact with special antigenic components of the cell membranes of blood platelets (platelets).

Normally, these blood cells perform a number of very important functions. physiological functions. They are necessary to ensure normal viscosity, as well as blood flow.

Latest results scientific research confirm that the structure of platelets in this hemorrhagic disease undergoes a number of changes. A pronounced granular apparatus appears in them, and the level of alpha-serotonin also increases significantly. A change in the total number of platelets affects the appearance of persistent changes that begin to occur in vascular wall. In response to this state the amount of platelet-derived growth factor increases.

All these disorders lead to the death of endothelial cells - the cells lining the inside blood vessels and giving them a specific “smoothness” necessary for uninterrupted blood flow. As a result of such pathological abnormalities, the first signs begin to appear in the child. hemorrhagic syndrome, which significantly worsens his well-being.

After suffering bacterial or viral infections antiplatelet antibodies usually appear after ½ -1 month. By their affiliation, they belong to the class of immunoglobulins G. This also determines the persistent preservation of the antibodies that appear in the blood in the future. In some cases, they can persist in the child throughout his life. The death of “spent” immune complexes occurs in the spleen.

Latest results conducted scientific studies made it possible to clarify why a child suffering from autoimmune thrombocytopenic purpura for a long time symptoms of increased bleeding remain. This is largely due to a decrease in serotonin levels. Normally, this substance is involved in the formation of a blood clot.

Kinds

Doctors distinguish several clinical forms of this hemorrhagic condition.

These include:

immune thrombocytopenia;
idiopathic.

Doctors also use another classification that allows them to divide various options thrombocytopenic purpura for various immune forms. These include:

Isoimmune. Most often occurs after blood transfusions. It can be congenital - when the mother and the unborn baby have an immune conflict regarding platelet antigens. May be transient in nature. This clinical form is often constantly relapsing.
Autoimmune. Occurs due to formation in the body large quantity platelet antibodies to your own blood platelets.

Heteroimmune. The development of this immune variant of the disease is often caused by taking certain groups of medications. An important role in this is played by the presence of individual hypersensitivity and immunity of individual chemical substances. This promotes development specific sign- purple rashes on the skin, which are the result of multiple hemorrhages.
Transimmune. This immune form of the disease develops, as a rule, as a result of an antigenic conflict and the accumulation of antiplatelet antibodies in a pregnant woman. They quite easily penetrate the fetal body through the placental blood flow system, leading to the development of hemorrhagic syndrome.

Symptoms

The severity of unfavorable clinical signs of the disease largely depends on how critical the decrease in platelets is in peripheral blood. Symptoms increase when the platelet level drops to 100,000/µl. A decrease to 50,000/µl leads to the appearance severe symptoms hemorrhagic syndrome.

This pathological condition is the most specific clinical sign of thrombocytopenic purpura. It is characterized by the appearance of numerous and varied hemorrhages that occur in a variety of anatomical areas.

Quite often, a sick child develops nose and gum bleeding; the most dangerous are hemorrhages in the brain and internal organs. This leads to disability of the sick baby.

Bleeding in the kidneys or urinary tract is manifested in a child by the presence of blood in the urine. With heavy bleeding, hematuria may occur - the appearance of a large number of red blood cells in the urinary sediment. Bleeding in gastrointestinal tract(especially in the distal parts of the intestine) cause the child to have black stools (melena). These Clinical signs are very unfavorable and require mandatory consultation with a doctor.

Hemorrhagic syndrome with thrombocytopenic purpura has several features. It is characterized by the asymmetry of the deviations that arise, as well as the complete spontaneity of their appearance. The severity of symptoms, as a rule, does not correspond to the intensity of the impact.

In some cases, adverse symptoms appear in a child even with a single dose of the drug or after suffering a common infection. Quite often, thrombocytopenic purpura occurs in an acute form.

Hemorrhages can be multiple and occur simultaneously (in a variety of internal organs). There are also quite unfavorable localizations internal bleeding. These include the kidneys, adrenal glands, brain, and spinal cord, heart, liver. Hemorrhages into these organs lead to severe impairment of vital functions.

Hemorrhage in the kidney

Subarachnoid hemorrhage in the brain

Thrombocytopenic purpura has certain characteristics. When palpating the internal organs, it can be noted that there is no enlargement of the spleen and liver. The lymph nodes also remain normal in size. These clinical signs differ significantly this pathology from many other hemorrhagic diseases. Quite often, the syndrome of increased bleeding remains only one manifestation of this disease.

How does it manifest in newborns?

The first symptoms in babies who have autoplatelet antibodies in their blood appear already in the first months of life. In infants, hemorrhagic syndrome can be expressed in different ways. The appearance of negative symptoms is influenced by the initial condition of the baby, as well as the presence of severe concomitant pathologies.

Thrombocytopenic purpura manifests itself in children by the occurrence of hemorrhages in the skin, mucous membranes, and also in internal organs.

Usually, the first symptoms that parents notice in a sick child are large bruises that suddenly appear on the skin. As a rule, there are no previous injuries or impacts before such elements appear on the skin. Development of hemorrhages into the cavity large joints is very dangerous, as it can lead to gait disturbances and pain when performing active movements.

Diagnostics

The disease can be suspected when a child develops various hemorrhages. Normally, babies do not have such manifestations. The appearance of bruises on the skin without connection with injury or blow should also motivate parents to consult a pediatrician with their baby. More accurate diagnosis A pediatric hematologist can diagnose it.

Diagnosis requires several laboratory tests. These studies help to establish the pathological variant of bleeding, as well as determine the severity of the child’s physiological disorders.

To identify excessive bleeding, a “pinch test” and a cuff test are performed. Examination using a pediatric tonometer cuff for measurement blood pressure is mandatory in the diagnosis of thrombocytopenic purpura.

Basic research, which is held for all children without exception, is general analysis blood. Given hemorrhagic disease characterized by a sharp decrease in platelet count. After viral infections, persistent lymphocytosis may be present in the blood for a long time. Carrying out a spinal puncture for thrombocytopenic purpura is only auxiliary. The result of a myelogram for this disease will show that the platelet count is normal.

In order to establish existing functional disorders a coagulogram analysis is performed. It helps determine the amount of fibrinogen, prothrombin time and others important criteria assessment of intravascular thrombus formation. The result is assessed by the attending physician.

Main medicines, which are prescribed by doctors to compensate for hemorrhagic syndrome, are glucocorticosteroids. They provide complex impact, including desensitizing, anti-inflammatory, immunosuppressive and antiallergic effects. The main drug prescribed during this period is prednisolone. The dosage of the hormone is determined individually, taking into account the age and weight of the baby.

When taking prednisolone, the number of platelets in the blood of a sick child gradually increases. Taking hormones helps reduce the amount of circulating antiplatelet antibodies, as well as reduce the formation of immune complexes that cause adverse symptoms.

To learn what thrombocytopenic purpura is, see the following video.

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What it is? Thrombocytopenic purpura (Werlhof's disease) is a pathology that is part of a large group of hemorrhagic (bloody) diathesis. It is characterized by the formation of subcutaneous hemorrhages (hemorrhages) and bleeding from the mucous membranes in children and adults.

Its manifestation is due to a qualitative or quantitative deficiency of platelets - thrombocytopenia, caused by insufficient secretion or increased breakdown of red blood cells. And thrombocytopathy, due to disturbances in the hemostasis process, leading to platelet deficiency and platelet dysfunction.

The first signs of thrombocytopenic purpura in children of both sexes may appear even before reaching puberty. At older ages (20-40 years), females are more susceptible to the disease.

We owe it all to dysfunction , responsible for many processes in the body, including hematopoiesis and immunity.

Types of thrombocytopenic purpura, photo

skin symptoms, photo 2

Development different types the disease is caused by several factors - according to the genesis, clinical manifestations and many other reasons. Includes independent and acquired (immune and non-immune factors):

Ideopathic – immune species thrombocytopenic purpura ( autoimmune disease) – an independent species, a primary manifestation provoked by antibodies that accelerate the destructive processes of platelet destruction;
Alloimmune (isoimmune and transimmune) – Rh-conflict type, neonatal pathology. Caused by platelet incompatibility (intrauterine mother-child conflict, infection by a sick mother, or due to the body’s immune reaction in response to multiple blood transfusions).
Hapten (heteroimmune) – a consequence of the secretion of antibodies to the effect external factors(viruses, drugs, etc.) that change the structural structure of platelets.

According to clinical characteristics, thrombocytopenic purpura occurs:

Acute course, manifesting itself mainly in children. Lasts no more than six months. After healing, no relapses are observed.
Chronic, characteristic of thrombocytopenic purpura in adults. The disease can be long-lasting – more than six months.
Recurrent, characterized by cyclical manifestation.

Clinical signs of purpura are expressed by isolated subcutaneous hemorrhages - the “dry form” and in combination with bleeding from mucous structures - the “wet form”.

Symptoms of thrombocytopenic purpura in adults

The development of chronic autoimmune thrombocytopenic purpura in adults begins gradually. The provoking factor in this case, as a rule, cannot be determined. There is a version about the connection between genesis and presence in the body chronic foci infections, or with harmful chemicals.

Erased (subclinical) a long period The disease does not allow it to be detected in a timely manner, which explains its accidental detection during a routine examination.

Hemorrhagic syndrome develops slowly, initially manifesting itself as single bruises and pinpoint subcutaneous hemorrhages, gradually developing to severe hemorrhages - hemoptysis, bloody vomiting and diarrhea, and blood in the urine.

The localization of subcutaneous hemorrhages is usually on the skin on the front of the limbs and torso. Often, at injection sites. Subcutaneous hemorrhages on the face, conjunctival mucosa and lips indicate a difficult process. An extremely severe clinical picture is characterized by hemorrhages in retina and brain with the development of severe anemia.

Presence on the surface of the oral mucosa filled with blood different sizes bullae and vesicles may be a harbinger of the development of hemorrhagic processes in the brain structure. Spontaneously, the disease in adults resolves extremely rarely.

Thrombocytopenic purpura in children, photo

Thrombocytopenic purpura in children photo

The most common type of idiopathic thrombocytopenic purpura is childhood It has acute development. In children from 2 to 6 years old, it begins two to four weeks after viral diseases or vaccination. In such children, the frequency of diseases does not differ by gender, only after reaching puberty, girls are susceptible to the disease twice as often.

The development of pathology is rapid, hemorrhages appear quickly and suddenly. Feature– multi-color coloring (polychrome). Subcutaneous hemorrhagic marks appear in various colors - scarlet, purple and greenish tones.

They can form spontaneously and asymmetrically, with point localization and larger ones - with multiple ecchymoses that can merge. They can be caused by squeezing and pinching when uncomfortable position in a dream, or the most minor bruise.

Typical symptoms of acute thrombocytopenic purpura are nasal and uterine hemorrhages, bleeding from the wound, extracted tooth. In severe processes, there are exacerbations of the child’s condition in the form of gastrointestinal or renal hemorrhages with the appearance of blood in the feces and urine.

Further development is complicated by posthemorrhagic anemia, bloody blisters in the oral cavity, profuse bleeding and damage to the retina.

Symptoms of hemorrhages in the brain may manifest itself:

Severe headaches and dizziness;
Sudden vomiting and convulsions;
Pathological neurological symptoms - drooping corners of the mouth, asymmetry of the eyes, impaired active motor functions and speeches.

Some children experience a slight increase in the size of the spleen (moderate splenomegaly). The child's temperature and general health, as a rule, does not change.

Teenage girls have long menstrual cycles. Duration acute process ITP – from one to two months, sometimes it can last up to six months. In a certain category of children, the disease may progress to the chronic stage.

With chronic thrombocytopenic purpura in adolescents and young children, spontaneous remission is possible. According to other signs, the disease in children is similar to thrombocytopenic purpura in adults.

Treatment of thrombocytopenic purpura, drugs

The complex selection of methods for the treatment of thrombocytopenic purpura is determined by the severity of clinical manifestations and the period of the disease. Includes conservative and surgical techniques. The basic principle of treatment is based on:

On mandatory hospitalization.
Complete rest until restoration of a stable hemogram of the platelet level.
A full-calorie diet (with fractional intake of chilled liquid food).
Prescribing hemostatic treatment - intravenous and drip injections of “Epsilonaminocaproic acid” and local funds in the form of sponges, fibrin films, tampons soaked in Thrombin, peroxide, or epsilonaminocaproic acid.
Drugs that improve platelet adhesion and the formation of blood clots that prevent bleeding - “Dacion”, “Calcium pantothenate”, “Adroxon” or “Etamsylate”.
Corticosteroids in the form of Prednisolone for profuse, recurring hemorrhages.
4-day course of drip administration of immunoglobulins.
Vitamin complexes and herbal medicine - rose hips, nettles, water pepper and many others.

In conditions that threaten the patient's life, or in the case of severe development of anemia, they resort to the method of blood transfusion. Only if conservative treatment of purpura of thrombocytopenic origin is ineffective, can it be used surgical method removal of the spleen - splenectomy.

In the most extreme cases, in combination with hormonal agents cytostatic drugs are prescribed - Imuran, Cyclophosphamide, Vinblastine, Mercaptopurine or Vincristine.

Forecast for thrombocytopenic purpura, for the most part – favorable. Mortality among sick adults and children is observed in exceptional cases. Patients with this diagnosis must be registered at the dispensary for at least five years.

A type of hemorrhagic diathesis, characterized by a deficiency of red blood platelets - platelets, most often caused by immune mechanisms. Signs of thrombocytopenic purpura are spontaneous, multiple, polymorphic hemorrhages in the skin and mucous membranes, as well as nasal, gingival, uterine and other bleeding. If thrombocytopenic purpura is suspected, anamnestic and clinical data, general blood count indicators, coagulograms, ELISA, microscopy of blood smears, and bone marrow puncture are assessed. IN medicinal purposes patients are prescribed corticosteroids, hemostatic drugs, cytostatic therapy, and splenectomy is performed.

General information

Thrombocytopenic purpura (Werlhof's disease, benign thrombocytopenia) is a hematological pathology characterized by a quantitative deficiency of platelets in the blood, accompanied by a tendency to bleeding and the development of hemorrhagic syndrome. With thrombocytopenic purpura, the level of blood platelets in the peripheral blood drops significantly below the physiological level - 150x10 9 / l with a normal or slightly increased number of megakaryocytes in the bone marrow. In terms of frequency of occurrence, thrombocytopenic purpura ranks first among other hemorrhagic diathesis. The disease usually manifests itself in childhood (with a peak in the early and preschool period). In adolescents and adults, pathology is detected 2-3 times more often among females.

The classification of thrombocytopenic purpura takes into account its etiological, pathogenetic and clinical features. There are several variants - idiopathic (Werlhof's disease), iso-, trans-, hetero- and autoimmune thrombocytopenic purpura, Werlhof's symptom complex (symptomatic thrombocytopenia).

According to the course, acute, chronic and recurrent forms are distinguished. The acute form is more typical for childhood, lasts up to 6 months with normalization of platelet levels in the blood, and has no relapses. The chronic form lasts more than 6 months and is more common in adult patients; recurrent - has a cyclical course with repetitions of episodes of thrombocytopenia after normalization of platelet levels.

Causes of thrombocytopenic purpura

In 45% of cases, idiopathic thrombocytopenic purpura occurs, developing spontaneously, without visible reasons. In 40% of cases of thrombocytopenia, various infectious diseases(viral or bacterial) transferred approximately 2-3 weeks earlier. In most cases, these are infections of the upper respiratory tract of nonspecific origin, in 20% - specific (chickenpox, measles, rubella, mumps, infectious mononucleosis, whooping cough). Thrombocytopenic purpura can complicate the course of malaria, typhoid fever, leishmaniasis, and septic endocarditis. Sometimes thrombocytopenic purpura appears against the background of immunization - active (vaccination) or passive (administration of γ - globulin). Thrombocytopenic purpura can be triggered by taking medications (barbiturates, estrogens, arsenic, mercury), prolonged exposure x-rays (radioactive isotopes), extensive surgical intervention, trauma, excessive insolation. Familial cases of the disease have been reported.

Most variants of thrombocytopenic purpura are of an immune nature and are associated with the production of antiplatelet antibodies (IgG). The formation of immune complexes on the surface of platelets leads to the rapid destruction of blood platelets, reducing their lifespan to several hours instead of 7-10 days normally.

The isoimmune form of thrombocytopenic purpura can be caused by the entry of “foreign” platelets into the blood during repeated blood or platelet transfusions, as well as antigenic incompatibility of platelets of the mother and fetus. The heteroimmune form develops when the antigenic structure of platelets is damaged by various agents (viruses, medications). The autoimmune variant of thrombocytopenic purpura is caused by the appearance of antibodies against one's own unchanged platelet antigens and is usually combined with other diseases of the same origin (SLE, autoimmune hemolytic anemia). The development of transimmune thrombocytopenia in newborns is provoked by antiplatelet autoantibodies passing through the placenta of a mother suffering from thrombocytopenic purpura.

Platelet deficiency in thrombocytopenic purpura may be associated with functional damage to megakaryocytes and a disruption in the process of shedding red blood platelets. For example, the Werlhof symptom complex is caused by the ineffectiveness of hematopoiesis in anemia (B-12 deficiency, aplastic), acute and chronic leukemia, systemic diseases hematopoietic organs (reticulosis), bone marrow metastases of malignant tumors.

With thrombocytopenic purpura, there is a disruption in the formation of thromboplastin and serotonin, a decrease in contractility and increased permeability of the capillary wall. This is associated with prolongation of bleeding time, disruption of thrombus formation and retraction processes. blood clot. During hemorrhagic exacerbations, the number of platelets decreases down to single cells in the drug; during the period of remission it is restored to a level below normal.

Symptoms of thrombocytopenic purpura

Thrombocytopenic purpura clinically manifests when the platelet count falls below 50x109/L, usually 2-3 weeks after exposure etiological factor. Petechial-spotted (bruised) type bleeding is characteristic. In patients with thrombocytopenic purpura, painless multiple hemorrhages appear under the skin, in the mucous membranes (“dry” version), as well as bleeding (“wet” version). They develop spontaneously (often at night) and their severity does not correspond to the strength of the traumatic impact.

Hemorrhagic rashes are polymorphic (from minor petechiae and ecchymoses to large bruises and bruises) and polychrome (from bright purplish-blue to pale yellow-green, depending on the time of appearance). Most often, hemorrhages occur on the anterior surface of the torso and limbs, rarely in the face and neck. Hemorrhages are also detected on the mucous membrane of the tonsils, soft and hard palate, conjunctiva and retina, eardrum, fatty tissue, parenchymal organs, serous membranes brain.

Intense bleeding is pathognomonic - nasal and gingival bleeding, bleeding after tooth extraction and tonsillectomy. Hemoptysis, bloody vomiting and diarrhea, and blood in the urine may occur. In women, uterine bleeding usually prevails in the form of menorrhagia and metrorrhagia, as well as ovulatory bleeding into the abdominal cavity with symptoms of ectopic pregnancy. Immediately before menstruation, skin hemorrhagic elements, nosebleeds and other bleeding appear. Body temperature remains normal, tachycardia is possible. In thrombocytopenic purpura there is moderate splenomegaly. With profuse bleeding, anemia of the internal organs, hyperplasia of the red bone marrow and megakaryocytes develops.

The medicinal form manifests itself soon after taking the drug, lasts from 1 week to 3 months with spontaneous recovery. Radiation thrombocytopenic purpura is characterized by severe hemorrhagic diathesis with the transition of the bone marrow to a hypo- and aplastic state. The infantile form (in children under 2 years of age) has an acute onset, severe, often chronic nature and severe thrombocytopenia (9/l).

During thrombocytopenic purpura, periods of hemorrhagic crisis, clinical and clinical-hematological remission are identified. During a hemorrhagic crisis, bleeding and laboratory changes are pronounced; during the period of clinical remission against the background of thrombocytopenia, hemorrhages do not appear. With complete remission, there is no bleeding or laboratory changes. With thrombocytopenic purpura with large blood loss, acute posthemorrhagic anemia is observed, with a long-term chronic form - chronic iron deficiency anemia.

The most dangerous complication - hemorrhage in the brain develops suddenly and progresses rapidly, accompanied by dizziness, headache, vomiting, convulsions, neurological disorders.

Diagnosis of thrombocytopenic purpura

The diagnosis of thrombocytopenic purpura is established by a hematologist taking into account the medical history, characteristics of the course and results laboratory research (clinical analysis blood and urine, coagulograms, ELISA, microscopy of blood smears, bone marrow puncture).

Thrombocytopenic purpura is indicated a sharp decline the number of platelets in the blood (9/l), an increase in bleeding time (>30 min.), prothrombin time and aPTT, a decrease in the degree or absence of clot retraction. The white blood cell count is usually within normal limits; anemia occurs with significant blood loss. At the height of the hemorrhagic crisis, positive endothelial tests (pinch, tourniquet, prick) are detected. A blood smear reveals an increase in size and a decrease in granularity of platelets. Red bone marrow preparations show normal or increased amount megakaryocytes, the presence of immature forms, lacing of platelets in few points. The autoimmune nature of purpura is confirmed by the presence of antiplatelet antibodies in the blood.). If the platelet level is 9/L, treatment is carried out without additional indications in a hospital setting.

Bleeding is stopped by the introduction of hemostatic drugs, applied topically hemostatic sponge. To curb immune reactions and reduce vascular permeability, corticosteroids are prescribed in a decreasing dose; hyperimmune globulins. In case of large blood losses, transfusions of plasma and washed red blood cells are possible. Platelet infusions are not indicated for thrombocytopenic purpura.

In patients with a chronic form with relapses heavy bleeding and hemorrhages in the vital important organs splenectomy is performed. It is possible to prescribe immunosuppressants (cytostatics). Treatment of thrombocytopenic purpura, if necessary, should be combined with therapy for the underlying disease.

In most cases, the prognosis of thrombocytopenic purpura is very favorable, complete recovery is possible in 75% of cases (in children - 90%). Complications (for example, hemorrhagic stroke) are observed in acute stage, creating a risk of death. In case of thrombocytopenic purpura, constant monitoring by a hematologist is required, drugs that affect the aggregation properties of platelets (acetylsalicylic acid, caffeine, barbiturates) are excluded. food allergens, caution is exercised when vaccinating children, and insolation is limited.

Hematologist

Higher education:

Hematologist

Samara State medical University(SamSMU, KMI)

Level of education - Specialist
1993-1999

Additional education:

"Hematology"

Russian Medical Academy Postgraduate Education

Thrombocytopenic purpura refers to blood diseases associated with a lack of platelets. It belongs to the group of hemorrhagic diathesis.

What is thrombocytopenic purpura?

A decrease in the number of platelets leads to a sharp decrease in blood clotting. Vessels suffer from lack of nutrition and are damaged inner layer, the permeability of the walls for red blood cells increases. The process is accompanied by a malfunction of the immune system, which begins to fight the cells own body- platelets.

Spontaneous bruising occurs on the body in the form of small red spots or bruises. They may look like pinpoint hemorrhages(petechiae), unite and form stripes or large spots. The disease gets its name – purpura – from the color of these spots. In fact, the color of hemorrhages can vary from purple-red to yellow-green.

Bleeding appears not only in the form skin rashes. Minor hemorrhages occur on the mucous membranes of the nose, in the oral cavity, on the sclera of the eyes, in adipose tissue, on internal organs and on the lining of the brain. Most often the disease manifests itself in childhood preschool age. Thrombocytopenic purpura in adults occurs just as often and mainly in women (their incidence is 3 times higher than in men).

Classification of thrombocytopenic purpura

Purpura is classified according to the form of the disease and the mechanism of its occurrence. According to the flow there are:

spicy;
chronic;
cyclically relapsing.

Acute thrombocytopenia most often occurs in childhood and lasts for six months. During this time, the level of platelets in the blood is restored to normal. It does not recur in the future. The chronic form affects adults and lasts more than 6 months. The recurrent form is manifested by alternating cycles of normalization and falling platelet levels.

Classification by mechanism of occurrence

According to the mechanism of occurrence, thrombocytopenic purpura is classified as follows:

idiopathic thrombocytopenic purpura or Werlhof's disease;
thrombotic thrombocytopenic purpura;
autoimmune thrombocytopenic purpura.

The etiology of the disease is different character. It can occur for no apparent reason. This type of purpura is called idiopathic and is not associated with other pathologies. This condition was first described by the doctor Paul Werlhof back in the 18th century, therefore the second name for idiopathic thrombocytopenic purpura is Werlhof's disease.

Thrombotic purpura

Thrombotic thrombocytopenic purpura is a type of disease that should be considered separately. It is severe and has a poor prognosis. Most often people between 30 and 40 years old get sick. It is also characterized by a decrease in platelets in the blood and the appearance of red spots on the skin.

However, its course is fundamentally different from thrombocytopenic purpura. Damaged platelets clump together and clog arterial capillaries, leading to organ ischemia. The brain and tissues of the lungs, heart and kidneys are affected. Thrombotic thrombocytopenic purpura initially takes an acute form and develops quite rapidly, accompanied by bleeding, fever, neurological symptoms - disorientation, tremor, blurred vision (double vision), convulsions, and other severe manifestations that can lead to coma. Always accompanied hemolytic anemia. Often the disease ends in the death of the patient from kidney failure.

Immune forms of thrombocytopenia

One of the reasons for platelet damage and death is an attack by one’s own immune cells. These attacks can be of different nature:

The transimmune form of the disease occurs in utero in the fetus, to which antiplatelet antibodies are transferred from the mother. In the first month of life, a child experiences a reduced number of platelets in the blood.
Isoimmune develops when someone else's blood is transfused.
The heteroimmune form is characterized by a change in the antigenic structure of platelets. At the same time, the cells of the immune system cease to recognize them and attack them as “strangers.” Doctors believe that these changes occur due to viral diseases or under the influence of certain medications. If the heteroimmune form of thrombocytopenia lasts more than 6 months, it is classified as autoimmune.
Autoimmune form. It also includes idiopathic thrombocytopenic purpura in adults, when the reason for the attack of one’s own immunity on platelets is unknown.

Immune thrombocytopenic purpura can occur at any age. It often takes on a recurrent or chronic nature. Women are more susceptible to it.

Causes of the disease

The exact causes of thrombocytopenic purpura are still a source of controversy among physicians. In almost 50% of cases it is diagnosed as idiopathic. In isolated cases, it is hereditary and is a consequence of impaired platelet production in the bone marrow. The most likely factors provoking the disease are:

viral diseases;
the effect of radiation;
metastases and bone marrow tumors;
mechanical damage to platelets during vascular replacement surgery;
reaction to vaccination and administration of gamma globulin;
administration of cytostatics during chemotherapy;
some oral contraceptives.

IN in rare cases the cause may be prolonged blood stagnation and pregnancy. Secondary purpura may appear as concomitant pathology for other diseases:

defeats connective tissue(systemic diseases);
leukemia;
hemophilia;
oncology with metastases in the bone marrow;
thrombocytopathies and some others.

Symptoms

Usually the disease develops rapidly. This is due to a catastrophic shortening of the lifespan of platelets. Its normal duration is 7 – 10 days. And under the influence of damaging factors it decreases to several hours. Symptoms are approximately the same for all forms of thrombocytopenic purpura, differing only in the intensity of manifestation:

the appearance of a pointed red rash or large subcutaneous hematomas;
hemorrhages due to skin injury (for example, after injection);
obvious bleeding from the mucous membranes;
hidden bleeding from eardrum, stomach and intestinal bleeding(the stool is stained with blood);
anemia developing against the background of constant blood loss;
nocturnal hemorrhages.

Possible coughing up of blood, bleeding in the brain and vitreous body of the eye. The body is covered with a rash only on the front. Its asymmetric localization is characteristic. The rash may be dry or bleeding. Autoimmune thrombocytopenia can cause an enlarged spleen. The rash is sometimes accompanied by a fever. Boys often experience nosebleeds, and girls often experience uterine bleeding.

Typically the disease progresses through three stages:

hemorrhagic crisis, when the platelet count decreases to a critical level;
clinical remission, in which external manifestations disappear, but a shift in laboratory tests remains;
Clinical and hematological remission, when they leave external symptoms and blood counts normalize.

Diagnostics

The primary diagnosis of thrombocytopenic purpura is a visual therapeutic examination external manifestations. Bleeding is also taken into account. Laboratory diagnostics includes a complete blood count for platelets, a bone marrow myelogram, and a biochemical blood test. In children, treatment is prescribed after examination and identification of symptoms. Held differential diagnosis secondary purpura.

Thrombotic purpura is additionally diagnosed by the number of leukocytes, recticulocytes and the state of erythrocytes (they are characterized by an altered shape). Indicators in the biochemical blood test increase (bilirubin, creatinine, urea, fibrinogen breakdown products appear).

Treatment

In the presence of hemorrhagic symptoms Treatment of thrombocytopenic purpura is carried out only in a hospital. For this disease, strict bed rest. First of all, the patient needs to stop bleeding and undergo therapy aimed at increasing platelet levels. The first drugs used for treatment are hemostatic drugs (Ascorutin, Thrombin). Then appointed hormone therapy(Prednisolone) and immunoglobulins. Taking corticosteroids continues for at least three months.

Transfusion of foreign platelets is almost never used in treatment, since it may not take root. At strong signs Anemia can be transfused with washed red blood cells - blood devoid of leukocytes and platelets. If the above methods do not produce results, resort to radical way– removal of the spleen. The operation is performed only when high risk its rupture and incessant bleeding.

After treatment, the patient is discharged and registered at the clinic. Parents are responsible for children. They are obliged to provide the child with a normal diet, periodic medical examination and minimize factors that provoke re-development diseases. Any allergenic products and protect the child from injury.

After treatment, adults are strictly contraindicated from taking blood thinners (for example, all types of aspirin), sleeping pills and sedatives, and caffeine. Dispensary registration for the disease lasts for at least two years.

All kinds of blood diseases (hemorrhagic) are a common phenomenon in modern medicine. About 50% of cases are thrombocytopenic purpura, the causes of which are numerous and not similar to each other. This disease affects the human body at any age.

Thrombocytopenic purpura - causes

Werlhof's disease or thrombocytopenic purpura, the causes of which are still not thoroughly known, is a phenomenon discovered by German scientists back in 1735. People call it “spotted disease” because in such patients, hemorrhages of various sizes appear here and there on the surface of the body.

The disease, in the overwhelming majority of cases, occurs in children from birth to adolescence, and then can completely disappear even without treatment. In children under two years of age, cases of purpura are more often recorded in boys. In adulthood, this diagnosis is made in 1-13 patients out of 100,000 people, with the majority of cases being women.

Forms of thrombocytopenic purpura

A blood disease such as thrombocytopenic purpura (Werlhof's disease) occurs when blood cells - platelets - are suppressed by their own immune system and die prematurely. Their lifespan, instead of 7-10 days, is several hours. The spleen is responsible for the production of autoimmune aggressors. The disease has several forms, which depend on the cause of the disease, but differ little in symptoms:

autoimmune or idiopathic;
heteroimmune;
transimmune.

Forms of thrombocytopenic idiopathic purpura, regardless of the reasons, are divided into:

spicy;
chronic (with frequent, rare or constant relapses).

Autoimmune thrombocytopenic purpura

In some cases, the body begins to work against itself. This is an autoimmune type of disease. Platelets are destroyed by the immune system, which perceives them as foreign cells. This type of disease sometimes occurs against the background of another autoimmune process in the body. Idiopathic thrombocytopenic purpura is characterized by the destruction of one's own platelets under the influence of certain factors. Sometimes this is a genetically transmitted pathology, although in most cases the reasons why the disease occurs are:

systemic lupus erythematosus;
autoimmune anemia of the hemolytic type;
allergic vasculitis;
chronic lymphocytic leukemia;
aggressive hepatitis;
glomerulonephritis.

Heteroimmune thrombocytopenic purpura

In childhood, the heteroimmune (non-immune) type of the disease, thrombotic thrombocytopenic purpura, occurs much more often than in adults. The good news is that children have a good prognosis for recovery. The causes of the disease are:

taking certain medications (paracetamol, furosemide, quinine, ampicillin, cephalosporins, beta blockers, etc.);
immunization (BCG vaccination, whooping cough, smallpox, influenza, polio);

infectious diseases.

Thrombocytopenic purpura - symptoms

Werlhof's disease, the symptoms of which are very eloquent, vary depending on the time of appearance of hemorrhages. That is, at first, bruises or, as doctors correctly call them - petechiae, are very pronounced, gradually reduce the intensity of color and turn into cyanotic, green tint, gradually turning yellow. At one point in time, all types of bruises can be on the body, which is a terrifying picture, but they are not painful, unlike bruises of traumatic origin in a healthy person.

Bruises are located mainly on the extremities, less often on the body, and very rarely on the face, but the mucous membranes are an exception. Thrombocytopenic purpura has similar symptoms from different people. They can occur either as a result of microtrauma or without a reason. Most hemorrhage occurs at night. In this case, the patient does not experience pain, with the exception of cerebral hemorrhage, when the patient urgently requires hospitalization. There are:

bleeding of mucous membranes (from the nose, gums, uterus);
internal bleeding (gastrointestinal, kidney, eye, brain).

Diagnosis of thrombocytopenic purpura

Werlhof's disease, the diagnosis of which does not end with the detection of symptoms, is based on the results of laboratory tests of patients and clinical examination. Doctors take a medical history when thrombocytopenic purpura first manifested itself. After being diagnosed accompanying illnesses and various types of studies are carried out from taking blood for analysis to conducting bleeding tests. These include:

Cuff test when a blood pressure cuff is placed on your arm for 10 minutes. If after this time there are formations under the cuff bloody dots, the test is positive. It is performed on children aged three years and older.
Tourniquet test when a medical tourniquet is applied to the arm, which after a while causes a bruise - this method is suitable for adult patients.

Thrombocytopenic purpura involves a series of tests that will show a reliable picture of the patient’s blood. These include blood tests for:

hemoglobin;
blood clotting rate;
platelet level;
absence or presence of blood clot retraction;
presence of antiplatelet cells in the blood.

In addition, in severe cases or when other types of diagnosis of thrombocytopenic purpura are unreliable, a bone marrow biopsy is performed - trephine biopsy. Using a special trephine instrument, a small puncture of the spine is made in the lumbar region to take small quantity material of bone, periosteum and itself cerebrospinal fluid for analysis.

Thrombocytopenic purpura - treatment

Blood diseases require a serious attitude, because it affects a lot at any age. Treatment of thrombocytopenic purpura is carried out both in a hospital setting (during the acute phase) and at home. It is imperative to provide the patient with maximum rest and bed rest to minimize the number of traumatic moments. Against this background, traditional medicinal methods together with traditional medicine. It is very important to adhere to a certain diet.

How curable is Werlhof's disease?

Depending on the age and at what stage Werlhof’s disease was diagnosed, the question of its curability will arise. In childhood, the prognosis is positive if the child’s disease is detected early and it is overcome within six months. After this time, the diagnosis becomes chronic, and sounds like hemorrhagic thrombocytopenia.

Adults are less fortunate - this condition can accompany them throughout their lives with intervals of remission and exacerbation. In some cases, chronic thrombocytopenic purpura can be cured by removing the spleen, although this is not 100% guaranteed. This organ destroys platelets and is an enemy of the body. The method is used as a last resort when other types of treatment have proven ineffective. They operate using both the abdominal method and laparoscopy.

Thrombocytopenic purpura - drugs

The goal of the course of treatment for Werlhof's disease is to maintain the level of platelets in the blood at the required level. Idiopathic thrombocytopenic purpura is treated, in most cases successfully, which allows achieving stable remission for long years. If neither treatment nor surgery helps, then the medication regimen is changed to a new one. For this use:

Prednisolone (Methylprednisolone) is a steroid hormone-containing drug to reduce the activity of the immune system. It is used in the first stages of treatment, before removal of the spleen.
Interferon a2 is used to suppress one's own antibodies when steroid drugs are powerless.
Immunoglobulin G is used before surgical intervention to increase platelet levels.
Immunosuppressants (Cyclophosphamide, Vincristine and Azathioprine) are used when the patient’s body does not accept other treatment. Antibodies in the blood are suppressed, leading to an increase in the number of platelets.
The drug Danazol, which stimulates the pituitary gland long-term use has a positive effect on the blood picture.

Thrombocytopenic purpura - treatment with folk remedies

Acute thrombocytopenic purpura is a blood disease, and therefore herbal remedies are successfully used to treat it. medicinal fees(decoctions) having hemostatic properties:

stinging nettle;
burnet;
viburnum bark;
chokeberry;
shepherd's purse;
chamomile;
yarrow.

Diet for thrombocytopenic purpura

There is no special nutrition for Werlhof's disease. It is important that dishes are consumed lukewarm or cool. Natural vegetables for thrombocytopenic purpura, like fruits, are very useful, but they should not cause allergies. The list of recommended products includes:

porridge – buckwheat, corn, oatmeal;
fruits and vegetables in the form of juices - carrot, apple, beetroot, pomegranate, tomato;
berries - currants, blackberries, raspberries;
seafood;
meat - beef.

The following should be excluded from the diet:

salty foods;
smoked meats;
seasonings and spices;
pickled vegetables;
fast food products;
carbonated drinks;
coffee;
alcohol.

Thrombocytopenic purpura - complications

Werlhof's disease, the complications of which are very dangerous, definitely requires strict medical supervision. Complications include:

cerebral hemorrhage;
gastric and kidney bleeding;
heavy menstrual bleeding in adolescence.

Thrombocytopenic purpura - prevention