Immunological disease thrombocytopenic purpura: features, signs, treatment. Collection of herbs for the treatment of thrombocytopenic purpura. Thrombocytopenic purpura in children and adults photo

Skin diseases affect children and adults with equal frequency. One of these ailments is thrombocytopenic purpura. Photo will be discussed in the article. The disease is due to its complex nature and special course. Let's look at how this process occurs in children, how often it occurs in adults, and what treatment methods are considered the most effective for eliminating the disease.

What is thrombocytopenic purpura?

Thrombocytopenic purpura, photos of which are presented below, is one of the types of hemorrhagic phenomenon - diathesis. It is characterized by a deficiency of red blood platelets - platelets, which is often caused by the influence of immune mechanisms. Signs of the disease include random effusions of blood into the skin, and they can be multiple or single. The disease is also accompanied by an increase in uterine, nasal and other blood flows. If progression is suspected of this disease, the task is to evaluate clinical data and other diagnostic information - ELISA, tests, smears and punctures.

This benign pathological is accompanied by the fact that there is a deficiency of platelets in the blood, and there is a tendency to increased bleeding and the development of hemorrhagic sign. In the presence of a disease in the peripheral blood system, a downward change in the level of blood platelets is observed. Among all hemorrhagic types diathesis, purpura occurs most often, mainly in preschool children age factor. In adults and adolescents, the pathological syndrome is less common and affects mainly women.

According to the course, the disease can be divided into acute and chronic forms. In the first case, the disease occurs in childhood and lasts six months, while platelets are normalized, and there are no relapses. In the second situation, the duration of the disease exceeds a period of 6 months and progresses in adults. The recurrent form has a cyclical course, with repeating functions of individual episodes.

Causes of the disease

In 45% of situations, the disease develops spontaneously. In 40% of cases, the development of this pathology is preceded by numerous viral and bacterial infectious phenomena that were experienced a couple of weeks before the onset. Typically, these factors include several diseases.

• chickenpox;
• measles, rubella;
• malaria;
• whooping cough;
• endocarditis.

Often the manifestation of the disease occurs as part of active or passive immunization. Several more factors that provoke the formation of the disease - intake medications, x-ray irradiation, impact chemical substances, surgical interventions, insolation.

Symptoms of thrombocytopenic purpura

The onset of any disease and manifestation is particularly acute. Sudden Appearance hemorrhages aggravates the situation and creates a characteristic of the disease appearance cover. Against the background of primary symptoms, additional signs – nosebleeds, worsening general condition, pale skin, sharp increase bodily temperature regime. If this acute process, full recovery without relapse usually occurs after 1 month. Against the backdrop of the onset of the disease, many children experience a noticeable increase in lymph nodes. By cause of lung In about 50% of children, the disease cannot be diagnosed.

If we are talking about chronic form illness, acute onset is not expected. First symptoms appear long before acute symptoms begin. Bleeding occurs in the area skin and looks like pinpoint hemorrhages. They aggravate the overall picture when the disease worsens. In first place among the formations is cutaneous purpura - or of an adult. Hemorrhagic manifestations usually appear abruptly, mainly at night.

In rare situations, the symptom can be “ bloody tears", which is due heavy bleeding from the eye area. This phenomenon is usually observed together with skin formations. Blood is flowing persistently and abundantly, against the background of this phenomenon anemia may occur. Gastrointestinal bleeding are less common, but in girls the process may be accompanied uterine pathologies and secretions. Rarely, purpura may cause hematomas and prolonged bleeding. Children suffering from the disease usually have no complaints, except that a couple of signs can be identified.

• General state of lethargy and apathy;
• increased fatigue;
• normal body temperature;
• normal size of the spleen and liver;
• sharp and severe irritability.

Chronic forms of the disease usually occur in children after 7 years of age. The course is wavy, with remissions and exacerbations alternating.

Thrombocytopenic purpura in children photo

Thrombocytopenic purpura, photos of which are presented in the article, has several forms and manifestations. The main signs of its presence in young children are hemorrhages and effusions into the mucous membranes and under the skin. The color of hemorrhages varies from red to green, and the size of the body ranges from small to large blisters and spots.

The manifestation is characterized by a lack of symmetry and logic of the sequence of formation. In children, the formations do not hurt or bother. When the stage of clinical remission occurs, the disease disappears and disappears completely.

Cutaneous hemorrhagic syndrome has several specific and characteristic features manifestations.

• The severity of the hemorrhagic symptom does not correspond to the traumatic impact. Formations can appear suddenly and unexpectedly.
• During the course of a childhood illness, polymorphism is observed, in which the variety and size of the rashes are different and multifaceted.
• Skin hemorrhage does not have favorite places localization. For example, it may appear on the body, face, and other parts of the body.

Most dangerous manifestation childhood illness is the appearance of hemorrhage in the sclera, which can give rise to the most severe complication– cerebral hemorrhage. The outcome of this course depends on the form and location of the pathology, as well as on the accepted therapeutic measures. Speaking about the prevalence of the disease, it can be noted that its frequency ranges from 10 to 125 cases per million population over an annual period.

Thrombocytopenic purpura in children treatment

Before you appoint effective techniques treatment, a study of the disease is carried out - a detailed diagnosis. There may be a decrease in the number of platelets in the blood and longest time bleeding. There is not always a correspondence between the duration this phenomenon and the degree of thrombocytopenia. Secondary indicators of the presence of the disease are altered properties of the blood, changes in hormones in it, as well as other substances.

Differential diagnosis

The disease must be differentiated from a number of other manifestations that are similar in their signs and symptomatic indicators.

• Acute leukemia – in in this case Metaplasia appears in the bone marrow area.
• SLE – to make a detailed diagnosis, it is necessary to take into account information from an immunological study.
• Thrombocytopathy involves a change in the content and level of platelets in the other direction.

Therapeutic activities for children

Thrombocytopenic purpura, photos of which are presented in the article, suggests no treatment if the forms of the disease are not severe. At moderate severity currents are shown therapeutic interventions, if there increased risk bleeding. Such phenomena are mainly characteristic of peptic ulcers, hypertension, duodenum. If the platelet level in the test reaches<20-30×10^9/л, то терапия назначается в стационарных условиях. Если больной человек страдает от хронической формы проявления заболевания, для которой характерны обильные течения крови и попадание жидкости в жизненно важные элементы, проводится спленэктомия. Ребенок во время геморрагического криза должен проводить время в постели.

1. If the therapy is pathogenetic in nature, glucocorticosteroids are prescribed at a dose of 2 mg/kg body weight per day orally, and immunosuppressants are used (cyclophosphamide, vincristine, if other methods are ineffective). All medications are specifically prescribed by a doctor.
2. To eliminate the symptoms and features of the disease, human Ig is used, and the child is given a dosage of 1 g per kg of weight for two days.
3. Danazol, which has an androgenic effect, is often used, as well as drugs from the interferon group - ROFERON-A, REAFERON, ANTI-A.
4. Drugs of the symptomatic group – aminocaproic acid, etamsylate.

Forecasting factors

In most situations, the prognosis is favorable. In children, full recovery occurs in 90% of cases, in adults – in 75%. In the acute stage During the course of the process, noticeable complications may arise in the form of a hemorrhagic stroke, and there is a high risk of death. When carrying out treatment, it is necessary that the sick person is constantly under the supervision of a hematologist, while it is important to exclude from the diet treatment agents that affect the properties of platelet aggregation. These drugs include caffeine, aspirin, barbiturate.

Thrombocytopenic purpura in adults photo

Along with other symptoms, this also occurs. The clinical picture is similar. Spots and formations on the skin do not bother a person. The only caveat is that thrombocytopenic purpura (photo can be seen in the article) among the adult population is 2-3 times more likely to affect the fair sex.

Thrombocytopenic purpura in adults treatment

The treatment complex for children and adults has many similarities, and all therapeutic methods are determined by how severe it is and how long it should last. Treatment consists of conservative therapy, which involves taking medications, as well as the use of surgical methods. Regardless of the manifestation of the disease, bed rest is an important part of recovery.

Drug treatment

Previously, remedies for treating the disease in children were described; in principle, they are all suitable for adults, but to simplify the situation and improve the condition, it is recommended to include in the diet hormonal drugs that help reduce vascular permeability and increase blood clotting. If there are multiple, as well as other symptoms, the use of several groups of remedies is effective.

1. PREDNISOONE 2 mg per kg of body weight per day is used, subsequently the dosage is reduced. Usually this therapy gives a tremendous effect; patients relapse after stopping hormones.
2. If the measures taken in adults do not produce results, use immunosuppressants. This imuran, 2-3 mg per kg of body weight per day. Duration of therapy is up to 5 months. Also wincry-steen 1-2 mg once a week.

The effect of such treatment can be detected after just a few weeks; over time, the dosage is reduced. Patients should also consume a vitamin complex, especially groups P and C. Such measures will make noticeable improvements in the properties of platelets. In addition, doctors prescribe calcium salts, aminocaproic acids, ATP, magnesium sulfate. Collections of natural folk remedies (nettle, rose hips, pepper, St. John's wort) are effective.

If conservative methods of therapy are not particularly effective, it comes to resorting to surgical removal of the spleen. Cytostatic agents are often prescribed to help reduce the number of harmful cells. But these drugs are used only in rare cases ( IMURAN, VINCRISTINE, CYCLOPHOSPHANE).

Diet for thrombocytopenic purpura

The main requirement that implies the possibility of quickly getting rid of the disease is adherence to a special diet. The patient's diet should be moderately high in calories and complete. Food is served chilled, it should be liquid and contain small portions. which was presented in the article, can quickly disappear if you eat the right foods.

Foods to reduce platelets

There is no specific treatment menu, but the main rule is to get nutritious food with proteins and essential vitamins. The benefit lies in the use of several product groups.

• Buckwheat porridge;
• peanuts and other nuts;
• fruits and juices from them;
• any vegetables;
• corn;
• beef liver;
• cereals;
• wheat and products made from it.

What to exclude from your diet

• spicy and salty foods;
• high fat foods;
• food with impurities and aromatic additives;
• hot spices and sauces;
• restaurant food and fast food products.

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About 40% of all hemorrhagic rashes are associated with thrombocytopenic purpura. Its prevalence ranges from 1 to 13 people per 100 thousand population, depending on the region.

Among all hemorrhagic diathesis, thrombocytopenic purpura ranks first in prevalence among preschool children; in adults, the syndrome is less common and affects mainly women.

What is thrombocytopenic purpura?

Thrombocytopenic purpura (Werlhof's disease, ITP, primary immune thrombocytopenia) is a disease characterized by a reduced level of platelets in the blood ().

The lifespan of platelets ranges from 7 to 10 days.

With thrombocytopenic purpura, the immune system suppresses its own blood cells, and they die within a few hours. The consequence of this is increased bleeding time.

Thrombocytopenic purpura is divided into acute, recurrent and chronic forms of the disease.

1. Mostly children suffer from the acute form, the disease lasts about 6 months, after recovery the level of platelets in the blood normalizes, there are no relapses.
2. The chronic form lasts more than 6 months and affects adults.
3. The recurrent form has a cyclical course: relapses are followed by periods of relief. Based on the frequency of exacerbations, the disease is divided into frequent, rare, and continuously recurrent.

Depending on the cause of the disease, thrombocytopenic purpura is divided into forms:

• isoimmune (alloimmune) thrombocytopenia occurs during fetal development and is associated with:
  • penetration of antiplatelet antibodies through the placenta from mother to child, a reduced level of platelets is observed in the prenatal period and in the first months after birth;
  • antiplatelet antibodies appear after blood transfusion;
• heteroimmune (hapten) thrombocytopenia occurs as a result of changes in platelet antigens; as a result, the immune system perceives its own blood cells as foreign. This reaction sometimes occurs as a result of a viral or bacterial infection, or after taking certain medications. If heteroimmune thrombocytopenia does not disappear within six months, then it is considered autoimmune.
• Autoimmune thrombocytopenia (AITP) has many forms, including idiopathic thrombocytopenic purpura (Werlhof's disease), the cause of which is unknown.

Causes

The reasons for the development of thrombocytic purpura still remain not fully established.

• 45% of cases of thrombocytic purpura are idiopathic (Werlhof's disease), that is, the causes have not been established.
• 40% of cases of this pathology are associated with a previous infectious disease and occur 2-3 weeks after recovery. Most often, an infection of viral origin is important (chickenpox, HIV infection, whooping cough, etc.), much less often - bacterial (malaria, typhoid fever, etc.)

Thrombocytopenic purpura can also occur due to:

• severe hypothermia;
• injuries;
• excessive insolation (ultraviolet radiation);
• radiation;
• complication after vaccine administration;
• taking certain medications (barbiturates, salicylic acid, antibiotics, estrogens, arsenic, mercury salts);
• disruption of the hematopoietic system;
• neoplasms localized in the bone marrow;
• vascular prosthetics due to mechanical damage to blood cells;
• taking certain oral contraceptives.

Risk factors

Thrombocytopenic purpura can occur in children and adults of any age, but there are factors that increase the risk of developing this condition:

• being female (thrombocytopenic purpura occurs three times more often in women than in men);
• a recent infectious disease increases the risk of developing the disease, especially in children;
• heredity (the disease in close relatives increases the risk of thrombocytopenia);
• frequent stress.

Symptoms of thrombocytopenic purpura

In Werlhof's disease, the main symptoms are hemorrhages.

Photo of idiopathic thrombocytopenic purpura

Werlhof's disease has three stages in its course:

• hemorrhagic crisis– the manifestations are pronounced; a blood test shows a reduced level of platelets;
• clinical remission– external signs of thrombocytopenia fade away, but its characteristic changes in blood tests are still present;
• clinical and hematological remission– the tests come back to normal, there are also no clinical signs of the disease.

The first signs of thrombocytopenia appear when the level of platelets in the blood decreases below 50*109/l, this occurs approximately 2-3 weeks after the factor that caused the disease was exposed.

Acute thrombocytopenic purpura

The disease begins suddenly: hemorrhages appear on the skin and mucous membranes (see photo above), bleeding begins, the general condition worsens, the skin becomes pale, and blood pressure drops.

Body temperature can reach 38 degrees. The lymph nodes become inflamed and painful.

Chronic thrombocytopenic purpura

Petechiae on the lateral surface of the tongue.

Signs of chronic disease

The main symptom of the disease is rash. They appear on the skin and mucous membranes, are painful, and vary in size.

Rashes can be of several types:

• petechiae (small dots);
• vibex (rash collects in groups or stripes);
• large spots, including petechiae and stripes.

The color of fresh rashes is purple. Faded rashes are yellow or green in color. The rash on the skin can be wet or dry.

In case of wet rashes, bleeding may occur, especially at night.

Common location: on the chest, abdomen, upper and lower extremities, rarely on the face and neck. At the same time, a rash appears on the mucous membranes.

Some locations are inaccessible for inspection without special equipment: the eardrum, serous membrane of the brain and other organs.

An important symptom of the disease is bleeding of varying intensity. The most commonly observed bleeding:

• nose bleed;
• from the gums and in places of extracted teeth;
• after surgery to remove tonsils;
• when coughing from the respiratory tract;
• from the digestive tract with vomit or urine;

Bleeding occurs either simultaneously with skin rashes or after.

The body temperature in chronic thrombocytopenic purpura is not elevated, sometimes there is a rapid heartbeat, and in children the lymph nodes become enlarged and painful.

Thrombotic form of idiopathic thrombocytopenic purpura.

This form of the disease is the most dangerous of all.

Characterized by an acute, spontaneous onset and malignant course.

Due to the formation of hyaline blood clots, the blood supply to various organs is disrupted.

Idiopathic thrombocytopenic purpura (ITP) of the thrombotic type is characterized by the following symptoms:

• hemorrhagic rash;
• fever;
• (it is this that causes death);
• convulsions, sensitivity disorders;
• joint pain;
• neurosis, confusion, coma;
• abdominal pain.

Diagnostics

To make a diagnosis of Werlhof's disease, a patient is interviewed and examined. Laboratory methods for examining blood, urine and the spinal cord are used to confirm the presence of the disease.

In chronic cases, hematological parameters may be within normal limits.

During the interview, a hematologist (a doctor specializing in blood diseases) finds out the presence of factors in the medical history that contribute to the development of thrombocytopenic purpura: viral infection, medication, vaccination, exposure to radiation, etc.

Examination of the patient reveals a characteristic sign of this disease - a hemorrhagic rash on the skin and mucous membranes. The doctor can also conduct a number of tests that make it possible to detect hemorrhages in the skin:

• Cuff test used in adults and children over 3 years of age. A blood pressure cuff is placed on the patient's arm; if pinpoint hemorrhages are detected after 10 minutes, the test is considered positive.
• Tourniquet test used only in adults. When a medical tourniquet is applied, hemorrhages appear at the point of pressure.
• "Pinch method"- after a small pinch, a bruise forms.

A general blood test reveals deviations in the level of hemoglobin (noted with significant blood loss) and platelets. shows the rate of blood clotting, the presence of antiplatelet antibodies, reduced or absent clot retraction. Red blood cells are detected.

In severe cases, a red bone marrow biopsy is performed. When examining a biopsy specimen, a normal or increased content of megakaryocytes and the presence of their immature forms are revealed.

In the case of characteristic clinical symptoms, treatment can be started immediately, without waiting for the results of laboratory tests.

Treatment of Werlhof's disease

If thrombocytopenic purpura does not cause complications, there is no significant bleeding, platelet counts in the blood are not lower than 50 * 109 / l, medical tactics consist of observation - no treatment is required.

When the platelet level decreases to 30-50*109/l, treatment is necessary for patients at risk for bleeding (arterial hypertension).

If the platelet level drops below 30*109/l, urgent hospitalization is required.

Conservative therapy

Conservative therapy includes the use of medications that suppress autoimmune processes and reduce vascular permeability:

• glucocorticosteroids (Prednisolone) have a treatment effect within 1-2 weeks;
• globulins (Immunoglobulin G), increases platelet levels;
• interferons (Interferon A2) are used when glucocorticosteroids are powerless;
• cytostatics (Cyclophosphamide, Imuran, Vinblastine, Vincristine and Azathioprine);
• vitamins PP and C, calcium salts, aminocaproic acid.

To stop external bleeding, a hemostatic sponge is used; in case of internal bleeding, drugs are administered to stop the bleeding.

In severe cases, plasmapheresis is used - transfusion of blood components and platelets.

Surgery

In some cases, idiopathic thrombocytopenic purpura does not respond to conservative therapy, then removal surgery is performed - splenectomy.

Significant improvement is immediately observed, but there is a risk of postoperative complications and the body's resistance to infectious diseases is significantly reduced.

Splenectomy is performed for a number of indications:

• the duration of the disease is more than 1 year, 2-3 periods of exacerbation, ineffectiveness of glucocorticosteroid therapy;
• contraindications to taking adrenocorticosteroids;
• relapse of thrombocytopenia after a course of drug therapy;
• severe course of idiopathic thrombocytopenic purpura, hemorrhages in vital organs.

Traditional medicine

To treat thrombocytic purpura, in addition to medications, medicinal plants with hemostatic properties are used. Among them:

• stinging nettle;
• horsetail;
• burnet;
• shepherd's purse;
• viburnum bark;
• sorrel;
• lemon balm;
• shepherd's purse;
• ginseng;
• Potentilla gossamer.

Diet

It is important that the food consumed is slightly warm or cool. Fresh vegetables and fruits are useful, but you need to make sure that they do not cause an allergic reaction.

Prohibited:

• salty foods;
• smoked;
• spices;
• fast food products;
• drinks: carbonated, alcoholic, coffee.

Forecast

In adults, thrombocytopenic purpura ends in complete recovery in 75% of cases, in children - in 90% of cases. The occurrence of serious complications is possible only in the acute period of the disease.

The likelihood of death with the thrombotic variant of thrombocytopenic purpura depends on the extent of the lesion and the degree of damage to the brain, cardiovascular system, kidneys and other organs.

Patients with a history of this pathology need constant medical supervision, exclusion of medications that negatively affect blood clotting, and a review of lifestyle and nutrition.

Prevention

Preventive measures for thrombocytopenic purpura are aimed at preventing exacerbations. They involve maintaining platelet and hemoglobin levels in the blood and include:

• exclusion of allergens in food (spicy, fried, alcohol);
• refusal of medications that affect platelet aggregation (ibuprofen, caffeinated drugs, sleeping pills, aspirin);
• abstaining from prolonged exposure to the sun;
• limit vaccination, refuse anti-influenza vaccine;
• avoiding contact with infectious patients;
• avoidance of traumatic sports;
• exclusion of strict diets;
• minimizing stressful situations.

After discharge from the hospital, the patient is registered at the dispensary at his place of residence - he is observed by a doctor for at least 2 years.

However, a large share of responsibility for health falls on the shoulders of the patient or his parents in the event of a child’s illness.

Given that thrombocytopenic purpura is common in children, it is very important to educate the entire family about how to prevent this disease.

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ABSTRACT

ON THE TOPIC OF: IDIOPATHIC THROMBOCYTOPENIC PURPURA. HEMORRHAGIC VASCULITIS

IDIOPATHIC THROMBOCYTOPENIC PURPURA (DISEASE VERLHOFA) ( Morbus maculosus Werlhofi )

Idiopathic thrombocytopenic purpura is one of the most common hemorrhagic diseases.

The etiology has not yet been established.

Pathogenesis. The disease is based on the destruction of platelets by antibodies synthesized in the organs of the immunocompetent system (primarily in the spleen, as well as in the bone marrow, liver and other organs containing lymphoid tissue). Antibodies, attaching to a certain part of the platelet membrane, form an immune complex with them, which is subsequently destroyed in the cells of the mononuclear phagocyte system, mainly in the spleen, which is the active zone of the macrophage system, and in severe cases, also in the liver and bone marrow. Possible intravascular platelet agglutination

Radioisotope methods have revealed a sharp shortening of the lifespan of platelets (up to several hours instead of 8-12 days).

Increased destruction of platelets leads to stimulation of thrombocytopoiesis by increasing the production of thrombopoietins. In idiopathic thrombocytopenic purpura, the content of megakaryocytes in the bone marrow is increased; the number of platelets formed per unit of time is not reduced, as previously thought, but, on the contrary, increased compared to the norm by 2-6 times. In this regard, for some time there is a balance between the production and destruction of platelets, which can be disrupted by various stressful situations - infection, intoxication, medications with hapten properties, as well as some physiological conditions - pregnancy, menopause, etc.

In the pathogenesis of bleeding in thrombocytopenic purpura, the leading role is played by thrombocytopenia and associated disturbances in the coagulation properties of blood and the permeability of the vascular wall. In this case, the first phase of blood coagulation—the formation of thromboplastin—is disrupted, as well as the retraction of the blood clot due to a deficiency of retractozyme.

Increased permeability of the vascular wall is also explained by thrombocytopenia. the absence of marginal standing of platelets, a violation of their angiotrophic function and a deficiency of serotonin, produced by platelets and having a powerful vasoconstrictor effect.

Clinic. The disease occurs most often at a young age and mainly among women. The main clinical symptoms are hemorrhages in the skin and bleeding from the mucous membranes, occurring either spontaneously or under the influence of minor injuries. Skin hemorrhages vary in size - from petechiae to large spots and even bruises, which are usually located on the anterior surface of the torso and limbs Depending on the duration hemorrhage, the initial purplish-red color gradually acquires various shades - blue, green and yellow, which gives the skin a characteristic appearance ("leopard skin").

A fairly common symptom of the disease is bleeding from the mucous membranes. The first place in frequency is occupied by bleeding from the nose and gums, and in women - menopause and metrorrhagia. Bleeding from the mucous membranes is usually combined with skin hemorrhages and is often multiple and profuse in nature, accompanied by the development of posthemorrhagic anemia.

In the initial period, they may be the only sign of the disease. Hemorrhages in the retina and other parts of the eye, in the brain, food canal, lungs and kidneys are also possible

An inconsistent symptom of the disease is a slight enlargement of the spleen, which, as a rule, cannot be palpated in thrombocytopenic purpura. Severe splenomegaly rather contradicts the diagnosis of Werlhof's disease. In most such cases, there is only symptomatic thrombocytopenia, accompanying splenopathy of various types.

The blood picture is characterized by a significant decrease in the number of platelets (in some cases until complete disappearance), changes in their morphological and functional properties (aniso- and poikilocytosis, decrease in granularity, decrease in the activity of lactate dehydrogenase, glucose-6-phosphate dehydrogenase, α-glycerophosphatase, decrease in aggregation and adhesive ability ).

Red blood counts and white blood cell counts are usually normal. Only in severe cases, with prolonged or profuse bleeding, does posthemorrhagic anemia develop.

Antibodies to platelets (thromboagglutinins and thrombolysins) can be detected in the blood.

When examining bone marrow puncture, an increased, less often normal, content of megakaryocytes is noted, around which platelets are usually absent. The latter circumstance is not associated with a decrease in the formation of blood platelets, as was previously interpreted, but with their accelerated entry into the blood.

Morphological changes in megakaryocytes (loss of granules, vacuolization, asynchrony of maturation) are often observed, explained by their increased platelet-forming function. In some cases of autoimmune thrombocytopenia, the number of megakaryocytes is reduced, which is apparently due to the presence of antibodies directed against megakaryocytes.

It should also be noted that there is a weakening or absence of blood clot retraction. If normally blood from a vein coagulates with the separation of serum after 2 hours, then with thrombocytopenic purpura the clot remains loose for 6 hours or even a day. Along with this, there is an increase in bleeding time (up to 10-20 minutes or more) with unchanged blood clotting time and a positive tourniquet symptom (Konchalovsky - Rumpel - Leede). However, it must be borne in mind that hemorrhagic tests are usually expressed only at the height of bleeding, almost disappearing in the remission stage.

The diagnosis is established on the basis of the multiple nature of hemorrhage, recurrence of the disease and a number of positive hemorrhagic tests (thrombocytopenia, slower retraction of a blood clot, increased bleeding time) with complete preservation and even hyperplasia of the megakaryocyte apparatus of the bone marrow.

Differential diagnosis is carried out with hemorrhagic vasculitis, hemophilia, hypoplastic anemia, acute leukemia, myelocarcinosis.

Thrombocytopenic purpura differs from hemorrhagic vasculitis and hemophilia by the presence of thrombocytopenia, the absence of articular manifestations, and normal blood clotting time (the latter is slowed down in hemophilia).

With hypoplastic anemia, in addition to a decrease in the number of platelets, anemia, leukopenia and granulonitopenia are observed, and in bone marrow puncture and trepanate - hypo- or aplasia.

Acute leukemia is characterized by the severe condition of patients, necrotic changes on the mucous membranes, anemia, the appearance of blast cells in the leukogram, and blast metaplasia of the bone marrow.

The distinctive signs of myelocarcinosis are the following: severe general condition, fever, emaciation, bone pain, bone damage with typical radiological changes, often accompanied by pathological fractures. A blood test determines anemia, the degree of which is not adequate to blood loss, often leukopenia, the appearance of young forms in the leukogram (metamyelocytes, myelocytes and even myeloblasts), and the presence of erythroblasts. Atypical cells can be detected in bone marrow punctate smears.

The course of the disease is mainly chronic, with alternating exacerbations and remissions of varying duration, during which hemorrhagic phenomena disappear, the number of platelets, clot retraction, etc. are often normalized. Along with this, chronic forms of the disease are observed with a monotonous course, constant hemorrhages on the skin or mucous membranes and thrombocytopenia.

In approximately 1/3 of patients, acute forms of the disease occur, characterized by a sudden onset, intense hemorrhagic manifestations, but in most cases ending with a rapid recovery.

The prognosis for thrombocytopenic purpura is generally favorable. In some cases, acute forms of the disease or its next relapses may be accompanied by profuse bleeding from the mucous membranes, followed by severe anemia, and sometimes by hemorrhage in the brain, which can lead to death.

Treatment should be aimed at relieving hemorrhagic manifestations, eliminating anemia and preventing relapses. To combat bleeding, vascular strengthening agents are prescribed (calcium preparations, ascorbic acid, rutin, ascorutin, chokeberry, etamsylate - dicinone, 1-2 tablets 3 times a day or 2-4 ml intramuscularly or intravenously, etc.). Aminocaproic acid is also used orally or intravenously, serotonin adipate 0.5-1 ml per day of 1 °/o solution intravenously or intramuscularly; locally - hemostatic sponge, thrombin, hemophobia, etc. In the presence of anemia and bleeding, blood transfusion with the shortest shelf life is recommended. For chronic posthemorrhagic anemia, iron supplements are indicated.

Transfusions of platelet mass should be limited only to urgent cases and not performed at all if the patient has antibodies to platelets due to a possible increase in bleeding.

Corticosteroids are widely used as pathogenetic therapy. The mechanism of their action is reduced to a decrease in the permeability of the vascular wall and inhibition of immune reactions, which is especially important in the immune form of thrombocytopenia, in which an increase in the number of platelets under the influence of corticosteroids is possible. Prednisolone is prescribed in a daily dose of 1 - 1.5 mg/kg. After stopping bleeding, the dose is gradually reduced to 20-25 mg per day. Maintenance therapy at these doses lasts 1-3 months. In case of a new exacerbation, a repeat course is carried out. Monitoring patients over the next year determines further therapeutic tactics. In case of ineffectiveness of conservative therapy, the presence of frequent exacerbations accompanied by heavy bleeding (especially in vital organs), and anemia, splenectomy is indicated, which gives remission in 75-80% of cases and is most effective in young people.

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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

The development of different types of disease is determined by several factors - according to genesis, clinical manifestations and many other reasons. Includes independent and acquired (immune and non-immune factors):

1. Ideopathic - immune type of thrombocytopenic purpura (autoimmune disease) - an independent type, the primary manifestation, provoked by antibodies that accelerate the destructive processes of platelet destruction;
2. 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).
3. Hapten (heteroimmune) – a consequence of the secretion of antibodies under the influence of external factors (viruses, drugs, etc.) that change the structural structure of platelets.

According to clinical characteristics, thrombocytopenic purpura is:

• 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 the genesis and the presence of chronic foci of infection in the body, or with harmful chemicals.

The erased (subclinical) long period of 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 the retina and brain with the development of severe anemia.

The presence of blood-filled bullae and vesicles of various sizes on the surface of the oral mucosa 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

Most often, idiopathic thrombocytopenic purpura in childhood has an 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. A characteristic feature is 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 constriction during an uncomfortable sleeping position, or by the most minor bruise.

Typical symptoms of acute thrombocytopenic purpura are nasal and uterine hemorrhages, bleeding from a wound, an 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 speech.

Some children experience a slight increase in the size of the spleen (moderate splenomegaly). The child’s temperature and general well-being, as a rule, do not change.

Teenage girls have long menstrual cycles. The duration of the acute ITP process is 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 agents in the form of sponges, fibrin films, tampons impregnated with “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 thrombocytopenic purpura is ineffective, a surgical method of removing the spleen - splenectomy - can be used.

In the most extreme cases, cytostatic drugs are prescribed in combination with hormonal drugs - 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.

Typically, thrombocytopenic purpura first develops in children aged 2-6 years (up to 10 years), regardless of gender. In adults, the disease is not as common, and women are more likely to suffer from it.

Characteristic of this disease is a decrease in the number of platelets in the blood serum below the level of 100 x10 9 /l against the background of sufficient formation in the bone marrow, and the presence of antibodies on the surface of platelets and in the blood that cause their destruction.

Depending on the duration and cyclicality of the course of the disease, there are several forms of thrombocytopenic purpura:
1. Spicy.
2. Chronic.
3. Recurrent.

The acute form is characterized by an increase in the level of blood platelets of more than 150x10 9 /l within 6 months from the date of development of the disease, in the absence of relapses (repeated cases of the disease) subsequently. If recovery of platelet levels is delayed for more than 6 months, a diagnosis of chronic thrombocytopenic purpura is made. When their number decreases below normal again after their recovery, recurrent thrombocytopenic purpura occurs.

Causes of thrombocytopenic purpura

• Rifampicin;
• Vancomycin;
• Bactrim;
• Carbamazepine;
• Diazepam;
• Sodium valproate;
• Methyldopa;
• Spironolactone;
• Levamisole;

Symptoms

Hemorrhages can occur not only on the mucous membranes of the oral cavity and tonsils, but also in the eardrum, vitreous body, sclera and fundus of the eye. Rarely, cerebral hemorrhage is possible, which significantly worsens the patient’s condition. This is preceded by the appearance of dizziness and headache, as well as bleeding in other organs.

When the platelet level decreases to less than 50x10 9 /l, nosebleeds and gum bleeding appear, which are more dangerous when a tooth is removed. In this case, bleeding occurs immediately and usually does not resume after it stops. In teenage girls with thrombocytopenic purpura, uterine bleeding during menstruation poses a certain danger.

Stages of thrombocytopenic purpura

Diagnostics

The examination complex includes the following diagnostic procedures:

• complete blood count with platelet count;
• determination of antiplatelet antibodies in the blood and Coombs test;
• bone marrow puncture;
• determination of APTT, prothrombin time, fibrinogen level;
• biochemical blood test (creatinine, urea, ALT, AST);
• Wasserman reaction, determination of antibodies to the Epstein-Barr virus, parvovirus in the blood.

Thrombocytopenic purpura in children

In children under 2 years of age, the infantile form of thrombocytopenic purpura is registered. In this case, the disease begins acutely, without the presence of a previous infection, and is extremely difficult: the platelet level drops below 20x10 9 /l, treatment is ineffective, and the risk of chronicity of the disease is very high.

The clinical manifestations of ITP depend on platelet levels. The onset of the disease is characterized by the appearance of spotty-bruised rashes on the skin and mild hemorrhages on the mucous membranes. When the platelet level decreases to less than 50 x10 9 /l, various bleedings may occur (nasal, gastrointestinal, uterine, renal). But most often, large “bruises” in places of contusions attract attention; there may be hematomas during intramuscular injections (injections). An enlarged spleen is characteristic. A general blood test records thrombocytopenia (decreased platelets), eosinophilia (increased number of eosinophils), anemia (decreased hemoglobin).

Treatment

Treatment of idiopathic thrombocytopenic purpura is aimed at reducing the production of antiplatelet antibodies and preventing their binding to platelets.

Diet for thrombocytopenic purpura

Drug therapy

• The general dose is prednisolone at a dose of 1-2 mg/kg per day for 21 days, then the dose is gradually reduced until completely discontinued. A repeat course is possible in a month.
• In high doses - prednisolone at a dose of 4-8 mg/kg per day is taken for a week, or methylprednisolone at a dose of 10-30 mg/kg per day, with rapid subsequent withdrawal of the drug, a second course is carried out after 1 week.
• "Pulse therapy" with hydrocortisone - 0.5 mg/kg per day, taken 4 days after 28 days (the course is 6 cycles).

With long-term use and individually, each patient may experience side effects from taking glucocorticoids: an increase in blood glucose levels and a decrease in potassium levels, stomach ulcers, decreased immunity, increased blood pressure, growth retardation.

2. Immunoglobulins for intravenous administration:

• Normal human immunoglobulin for intravenous administration;
• Intraglobin F;
• Octagam;
• Sandoglobulin;
• Venoglobulin, etc.

With the use of immunoglobulins, headaches, allergic reactions, increased body temperature to high levels and chills may occur. To reduce the severity of undesirable effects, Paracetamol and Diphenhydramine are prescribed orally, and Dexamethasone intravenously.

3. Interferon alpha.
Indicated for chronic purpura in case of ineffective treatment with glucocorticoids. 2x106 units of interferon-alpha are injected under the skin or into the muscle for a month, 3 times a week, every other day.

Often during treatment with interferon appear