Why does hemorrhagic purpura occur and why is it dangerous? Thrombocytopenic purpura: forms, symptoms in children and adults, treatment

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

A 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 their 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 cyclicity of the course of the disease, there are several forms of thrombocytopenic purpura:
1. Acute.
2. Chronic.
3. Recurrent.

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

Causes of thrombocytopenic purpura

• Rifampicin;
• Vancomycin;
• Bactrim;
• Carbomazepine;
• diazepam;
• sodium valproate;
• Methyldopa;
• Spironolactone;
• Levamisole;

Symptoms

Hemorrhages can be not only on the mucous membranes of the oral cavity and tonsils, but also in the tympanic membrane, vitreous body, sclera and fundus. 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.

With a decrease in the level of platelets less than 50x10 9 /l, nosebleeds, gingival 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 adolescent girls with thrombocytopenic purpura, uterine bleeding during menstruation is a certain danger.

Stages of the course of thrombocytopenic purpura

Diagnostics

The examination complex includes the following diagnostic procedures:

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

Thrombocytopenic purpura in children

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

The clinical manifestations of ITP depend on the level of platelets. The onset of the disease is characterized by the appearance of spotty-bruising rashes on the skin and unexpressed hemorrhages on the mucous membranes. With a decrease in the level of platelets less than 50 x10 9 /l, various bleeding may occur (nasal, gastrointestinal, uterine, renal). But most often attention is drawn to large "bruises" in places of bruises, there may be hematomas during intramuscular injections (shots). Enlargement of the spleen is characteristic. In the general blood test, thrombocytopenia (a decrease in platelets), eosinophilia (an increase in the number of eosinophils), anemia (a decrease in the amount of hemoglobin) is recorded.

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

Medical therapy

• In the total dose - prednisolone at a dose of 1-2 mg / kg per day for 21 days, then the dose is gradually reduced until it is completely canceled. A second 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 and a decrease in potassium levels, stomach ulcers, decreased immunity, increased blood pressure, growth retardation.

2. Immunoglobulins for intravenous administration:

• Human normal immunoglobulin for intravenous administration;
• Intraglobin F;
• Octagam;
• Sandoglobulin;
• Venoglobulin, etc.

Against the background of the use of immunoglobulins, a headache, an allergic reaction, an increase in body temperature to high numbers and chills may occur. To reduce the severity of undesirable effects, it is necessary to remember the possibility of tissue necrosis with the introduction of rose periwinkle alkaloids subcutaneously. Perhaps a violation of nerve conduction, the appearance of foci of baldness (alopecia).

5. Danazol.
Apply the drug through the mouth, at a dose of 10-20 mg / kg per day. It is necessary to divide the dose into 3 doses, the course is 3 months.
Shows a toxic effect on the liver, possibly weight gain during treatment, the appearance of excessive hair growth (hirsutism).

6. Cyclophosphamide.
It is prescribed through the mouth, at a dose of 1-2 mg / kg per day. The therapeutic effect appears after 7-10 days.
Like Danazol, this drug is toxic to liver cells and can lead to weight gain and alopecia. Causes hemorrhagic cystitis in some patients.

7. Azathioprine
Take 200-400 mg per day, for 3 months to six months, after the end of treatment, maintenance therapy is necessary.
While taking the drug, patients are worried about nausea, loss of appetite, vomiting.

Infusion (infusion) of platelets

Splenectomy

Plasmapheresis

• exacerbation of stomach ulcers;
• unstopped bleeding;
• hemoglobin level less than 90g / l with iron deficiency anemia;
• oncological diseases.

A type of hemorrhagic diathesis, characterized by a deficiency of red blood platelets - platelets, 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, indicators of a general blood test, coagulogram, ELISA, microscopy of blood smears, and bone marrow puncture are evaluated. For therapeutic purposes, patients are prescribed corticosteroid, hemostatic drugs, cytostatic therapy, 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, the development of hemorrhagic syndrome. With thrombocytopenic purpura, the level of 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 in childhood (with a peak in the early and preschool period). In adolescents and adults, pathology is 2-3 times more likely to be detected among females.

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

Along 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 the level of platelets in the blood, has no relapses. The chronic form lasts more than 6 months, is more common in adult patients; recurrent - has a cyclic course with repetitions of episodes of thrombocytopenia after normalization of platelet levels.

Causes of thrombocytopenic purpura

In 45% of cases, there is idiopathic thrombocytopenic purpura, which develops spontaneously, for no apparent reason. In 40% of cases, thrombocytopenia is preceded by various infectious diseases (viral or bacterial) transferred approximately 2-3 weeks before. In most cases, these are infections of the upper respiratory tract of non-specific origin, in 20% - specific (chicken pox, measles, rubella, mumps, infectious mononucleosis, whooping cough). Thrombocytopenic purpura can complicate the course of malaria, typhoid fever, leishmaniasis, and septic endocarditis. Sometimes thrombocytopenic purpura manifests itself against the background of immunization - active (vaccination) or passive (introduction of γ - globulin). Thrombocytopenic purpura can be triggered by medication (barbiturates, estrogens, arsenic, mercury), prolonged exposure to X-rays (radioactive isotopes), extensive surgery, trauma, excessive insolation. Familial cases have been reported.

Most variants of thrombocytopenic purpura are immune in 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 platelets, reducing their lifespan to several hours instead of 7-10 days normally.

The isoimmune form of thrombocytopenic purpura may be due to the entry into the blood of "foreign" platelets during repeated blood transfusions or platelet mass, as well as antigenic incompatibility of maternal and fetal platelets. The heteroimmune form develops when the antigenic structure of platelets is damaged by various agents (viruses, drugs). The autoimmune variant of thrombocytopenic purpura is caused by the appearance of antibodies against its 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 with thrombocytopenic purpura.

Platelet deficiency in thrombocytopenic purpura may be associated with a functional lesion of megakaryocytes, a violation of the process of lacing of blood red platelets. For example, Verlhof's symptom complex is due to the ineffectiveness of hematopoiesis in anemia (B-12 deficient, aplastic), acute and chronic leukemia, systemic diseases of the hematopoietic organs (reticulosis), and bone marrow metastases of malignant tumors.

With thrombocytopenic purpura, there is a violation of the formation of thromboplastin and serotonin, a decrease in contractility and an increase in the permeability of the capillary wall. This is associated with prolongation of bleeding time, violation of the processes of thrombosis and retraction of the blood clot. With hemorrhagic exacerbations, the number of platelets decreases down to single cells in the preparation, during the period of remission it is restored to a level below the norm.

Symptoms of thrombocytopenic purpura

Thrombocytopenic purpura is clinically manifested when the platelet level falls below 50x10 9 /l, usually 2-3 weeks after exposure to the etiological factor. Bleeding of the petechial-spotted (bruising) type is characteristic. In patients with thrombocytopenic purpura, painless multiple hemorrhages appear under the skin, in the mucous membranes ("dry" variant), as well as bleeding ("wet" variant). They develop spontaneously (often at night) and their severity does not correspond to the strength of the traumatic impact.

Hemorrhagic eruptions are polymorphic (from minor petechiae and ecchymosis to large bruises and bruises) and polychromic (from bright purple-blue to pale yellow-green, depending on the time of appearance). Most often, hemorrhages occur on the anterior surface of the trunk and extremities, rarely in the face and neck. Hemorrhages are also determined on the mucous membrane of the tonsils, soft and hard palate, conjunctiva and retina, eardrum, in fatty tissue, parenchymal organs, serous membranes of the brain.

Intense bleeding is pathognomonic - nasal and gingival, bleeding after tooth extraction and tonsillectomy. There may be hemoptysis, bloody vomiting and diarrhea, blood in the urine. 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, nasal and other bleeding appear. Body temperature remains normal, tachycardia is possible. Thrombocytopenic purpura has moderate splenomegaly. With profuse bleeding, anemia of internal organs develops, hyperplasia of the red bone marrow and megakaryocytes.

The drug form manifests shortly 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, and severe thrombocytopenia (9/l).

During thrombocytopenic purpura, periods of hemorrhagic crisis, clinical and clinical-hematological remission are revealed. In 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 formidable complication - cerebral hemorrhage 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 anamnesis, the course and the results of laboratory tests (clinical analysis of blood and urine, coagulogram, ELISA, microscopy of blood smears, bone marrow puncture).

Thrombocytopenic purpura is indicated by a sharp decrease in 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 number of leukocytes is usually within the normal range, anemia appears with significant blood loss. At the height of the hemorrhagic crisis, positive endothelial tests (pinch, tourniquet, prick test) are detected. In a blood smear, an increase in size and a decrease in platelet granularity are determined. In preparations of red bone marrow, a normal or increased number of megakaryocytes, the presence of immature forms, and platelet lacing at small points are found. The autoimmune nature of purpura is confirmed by the presence of antiplatelet antibodies in the blood.

Thrombocytopenic purpura is differentiated from aplastic or infiltrative bone marrow processes, acute leukemia, thrombocytopathy, SLE, hemophilia, hemorrhagic vasculitis, hypo- and dysfibrinogenemia, juvenile uterine bleeding.

Treatment and prognosis of thrombocytopenic purpura

With thrombocytopenic purpura with isolated thrombocytopenia (platelets> 50x10 9 / l) without hemorrhagic syndrome, treatment is not carried out; with moderate thrombocytopenia (30-50 x10 9 /l), drug therapy is indicated in case of an increased risk of bleeding (arterial hypertension, gastric ulcer and duodenal ulcer). At a platelet level of 9/l, treatment is carried out without additional indications in a hospital setting.

Bleeding is stopped by the introduction of hemostatic drugs, a hemostatic sponge is applied topically. To curb immune responses and reduce vascular permeability, corticosteroids are prescribed at a lower dose; hyperimmune globulins. With large blood loss, transfusions of plasma and washed erythrocytes are possible. Platelet mass infusions are not indicated for thrombocytopenic purpura.

In patients with a chronic form with relapses of heavy bleeding and hemorrhages in vital organs, splenectomy is performed. Perhaps the appointment of 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 - in 90%). Complications (for example, hemorrhagic stroke) are observed in the acute stage, creating a risk of death. With thrombocytopenic purpura, constant monitoring by a hematologist is required, drugs that affect the aggregation properties of platelets (acetylsalicylic acid, caffeine, barbiturates), food allergens are excluded, caution is shown when vaccinating children, insolation is limited.

Thrombocytopenic purpura is one of the most common types of hemorrhagic diathesis.

Some of the types, such as thrombotic
thrombocytopenic purpura, have fulminant
malignant course and in the vast majority of cases end in the death of the patient.

The mechanism of development of thrombocytopenic purpura

The origin and development of purpura is based on violations of the platelet component of the blood composition, in which - 150 * 109 / l.

This can occur with increased destruction of platelets for various reasons, most often associated with the immunological status of the patient.

More often, thrombocytopenic purpura is diagnosed in children 2-7 years old, but can occur both in adults and in adults.

Before reaching the age of 10, boys and girls get sick equally, after 10 years, the disease is detected much more often in girls.

Classification

Depending on the etiology (cause of occurrence) and pathogenesis (pathological mechanism of occurrence and development), it is customary to distinguish several varieties of thrombocytopenic purpura:

1. (for no apparent reason);
2. isoimmune, which arose as the body's own response to repeated blood transfusions or. Sometimes occurs during pregnancy;
3. innate immune arises as a response to the incompatibility of the blood of the mother and fetus. Often, by 4-5 months, the child self-heals;
4. autoimmune - the result of the body's own response to certain diseases and pathological conditions (systemic lupus erythematosus, anemia, acute leukemia, etc.);
5. symptomatic - a temporary phenomenon that occurs with a deficiency of vitamin B12, with certain infectious diseases, radiation sickness, taking a number of potent pharmaceuticals, etc.

The reasons

Most often, purpura occurs against the background and as a consequence of past viral diseases (flu, mumps - "mumps", whooping cough, chicken pox, rubella, measles, chickenpox, etc.).

There have been cases when preventive vaccination against influenza was the trigger for the appearance of thrombocytopenic purpura in children.

Often there is a combination with endocarditis, leishmaniasis, malaria, typhoid fever. In this case, thrombocytopenic purpura aggravates the course of the underlying infectious disease.

Immunological forms

Almost all types of immunological purpura are associated with the production of antiplatelet antibodies (IgG).

Due to the formation of immune complexes on the surface of platelet plates, they are rapidly destroyed.

Thrombocytopenic purpura is characterized by:

• decrease in platelet count in blood tests (<50*109/л);
• increased bleeding time (more than 30 minutes);
• the leukocyte formula remains within the normal range. The development of anemia is possible with severe blood loss;
• microscopy of blood smears reveals an increase in platelets in size and a decrease in granularity;
• in preparations of bone marrow punctate, a large number of karyocytes, platelet lacing are detected.

Differential diagnosis is carried out with the following diseases:

• various pathological processes in the bone marrow;
• acute leukemia;
• hemophilia;
• hemorrhagic vasculitis;
• dysfibrinomegaly;
• juvenile (childhood) uterine bleeding.

Treatment of thrombocytopenic purpura

With indirect or mild clinical manifestations and a platelet level of more than 50 * 109 / l, special treatment is not carried out.

At a level of 30-50 * 109 / l, therapy is carried out aimed at preventing bleeding.

If the platelet count is less than 30*109/l, the patient needs hospital treatment.

Hemostatic drugs are used to stop bleeding. To suppress the autoimmune response, hyperimmune globulins and corticosteroids are prescribed.

In case of significant blood loss, an infusion of erythrocyte mass is indicated. Platelet transfusions are excluded. In some cases, cytostatics are prescribed.

Treatment of the chronic form of thrombocytopenic purpura should be carried out in parallel with the treatment of the underlying disease (with secondary or symptomatic variants of occurrence and development).

In rare cases, with ineffective medical treatment of a chronic and severe form of the disease, surgical treatment is indicated - splenectomy (removal of the spleen).

Rehabilitation

Forecast

Most often, the prognosis is favorable. The disease resolves on its own or after specific therapy in 75% of cases without complications.

Hemorrhagic stroke is rare, but dangerous in the acute phase of the disease, a fatal outcome is possible.

The disease is curable, most often there is a complete recovery after timely and.

The disease is more often observed in preschool age, less often in early childhood and school. Boys and girls get sick equally often. The pathogenesis of thrombocytopenic purpura as an independent disease is the same as in adults. Symptomatic thrombocytopenic purpura occurs in children with infections, chronic tonsillogenic intoxication, diseases of the blood system, kidneys, as well as under the influence of drugs (, barbiturates) and radiation factors.

The main symptom of the disease is subcutaneous hemorrhage, different in size and color, located asymmetrically, usually occurring without cause, sometimes after a febrile illness, more often at night. Children often have nosebleeds, in addition, in girls in the puberty period - uterine, in - bleeding from. Changes in blood composition are the same as in adults.

Treatment. Calcium preparations are prescribed (for example, calcium gluconate before meals 2-3 times a day for children under 1 year old, 0.5 g each, from 2 to 4 years old - 1 g each, from 5 to 6 years old - 1-1.5 g each, from 7 to 9 years old - 1.5-2 g each, from 10 to 14 years old - 2-3 g each), large doses of ascorbic acid (up to 1 g) and vitamin P (for example, 0.02 g 3 times a day day). Treatment of immune forms of thrombocytopenic purpura is carried out for 1-2 months. corticosteroids at the rate of 1.5 mg / kg per day, while 2/3 of the dose is prescribed in the morning (at 7 and 10 hours). With profuse bleeding, transfusions of fresh blood 70-100 ml, plasma 30-50 ml, platelet mass 50-100 ml are indicated. If there is no effect within 6 months, as well as with a threat of cerebral hemorrhage, it is indicated. With symptomatic thrombocytopenic purpura, the underlying disease is treated.

Thrombocytopenic purpura in children often occurs with thrombocytopathy, which ranks first in frequency among hemorrhagic diathesis in children. Symptomatic thrombocytopenic purpura (Werlhof's symptom complex) occurs in children with the same diseases and intoxications as in adults.

Thrombocytopenic purpura can be congenital or occur during any period of childhood, including neonatal and early childhood. Most often the disease is detected clinically at the age of 3-14 years. Since puberty, girls are more likely to get sick. Heredity plays a role, unlike hemophilia, not related to sex. Often in families with thrombocytopenic purpura, hemorrhagic diathesis is noted.

The etiology of "idiopathic" thrombocytopenic purpura in children is the same as in adults. A significant role in the origin of thrombocytopenic purpura in children is played by metabolic disorders leading to increased vascular permeability, dysfunction of the reticuloendothelial system, and vascular endothelial insufficiency. The importance of neuroendocrine factors in the pathogenesis of thrombocytopenic purpura in children is indicated by an increase in the development of the disease in girls during puberty, the relationship of the occurrence of purpura with stress reactions.

"Idiopathic" thrombocytopenic purpura in newborns is rare (A. F. Tur). It is associated with the transfer of antiplatelet antibodies from mother to child (isoimmune form). Diagnosis of immune thrombocytopenic purpura is possible only in the laboratory. A peculiar form of thrombocytopenic purpura in newborns is congenital hypoplastic thrombocytopenia, in which there is a congenital anomaly of the bone marrow, characterized by the absence of megakaryocytes in it. This form is very severe and ends lethally in the first months of life. More often with thrombocytopenic purpura of newborns, despite the appearance of clinical symptoms soon after birth, the hemorrhagic syndrome gradually disappears after a few weeks or months.

Clinical picture and course. The main symptoms of thrombocytopenic purpura in children are hemorrhages and bleeding. Hemorrhages can be superficial and deeper. Different size and color, irregular shape of skin hemorrhages give the impression of a "leopard skin". Characterized by the absence of hemorrhages in the joints. Occasionally, bullous-hemorrhagic rashes occur, indicating the severity of the lesion. Skin hemorrhages may be accompanied by hemorrhages on the mucous membranes, sometimes in the conjunctiva of the eyes.

Thrombocytopenic purpura in children is characterized by bleeding, most often nasal, and in girls in the pubertal period - uterine bleeding. In newborns, the only manifestation of thrombocytopenic purpura may be bleeding from the umbilicus. A big rarity in thrombocytopenic purpura is bleeding from the digestive tract, respiratory and urinary tract. Profuse fatal bleeding is also exceptionally rare.

The well-being of children can remain satisfactory for a long time, but frequent and prolonged bleeding weakens the child. Often, thrombocytopenic purpura is accompanied by subfebrile, sometimes even higher temperature. An enlarged and slightly compacted spleen is observed in 30-35% of sick children. Hepatomegaly is even less common.

Changes in the blood picture and indicators of hemorrhagic syndrome in thrombocytopenic purpura in children are similar to changes in adults. In hemorrhagic crises, most children have a decrease in platelets below the "critical" number (30,000). The bone marrow in thrombocytopenic purpura in children may not be changed.

The acute course of thrombocytopenic purpura is rarely observed, mainly in symptomatic forms, as well as in newborns and children in the first months of life. Much more often in children there is a chronic (over 6 months) undulating or continuous course of the disease. The interval between crises can reach several years (according to V. N. Zasukhina, up to 10-15 years), but more often does not exceed several weeks or months.

The most serious, although rare, manifestations of thrombocytopenic purpura in children are hemorrhages in the brain, sensory organs, as well as profuse bleeding, which can even lead to death (hemorrhages in the central nervous system or adrenal glands).

Glanzmann's hemorrhagic thrombasthenia (thrombocytoasthenia, vascular purpura with a lack of retractozyme) is a familial hereditary disease transmitted by a dominant type with a clinical picture similar to Werlhof's disease. The course of the disease is usually milder. Hematological examination showed no thrombocytopenia, the main symptom of Werlhof's disease. Prolonged bleeding time and impaired retraction of the blood clot are characteristic. In the pathogenesis of thrombasthenia, qualitative inferiority, degeneration of platelets and a lack of retractozyme in them are of primary importance. The disease is also characterized by a decrease in blood serotonin, a violation of thromboplastin formation and a decrease in the consumption of blood prothrombin. Glanzmann's thrombasthenia occurs mainly in children.

Capillaropathic purpura (synonym: von Willebrand-Jurgens disease, angiohemophilia, hereditary pseudohemophilia, hemorrhagic capillaropathy, constitutional thrombopathy, athrombocytopenic purpura) is characterized by a systemic lesion of the capillary network. Unlike true thrombocytopenic purpura, with capillaropathy, it is not hemorrhages that come to the fore, but bleeding (vascular hemophilia). There is a systemic pathological dilatation of capillaries, a decrease in the content of serotonin, some coagulation factors (V, VII, VIII, IX) and platelet factors. The most significant is a significant prolongation of bleeding time.

Platelets were not changed quantitatively and qualitatively, retraction of the blood clot was normal. The disease develops at an early age in children of both sexes, has a cyclic course; with age, bleeding in capillary purpura may decrease.

In the differential diagnosis of thrombocytopenic purpura, one should also keep in mind Shenlein-Genoch disease and other vasculitis, hemophilia, pseudohemophilia, dysovarian purpura in girls, scurvy, Waterhouse-Friderichsen syndrome, aplastic anemia, leukemia, multiple myeloma, etc.

Combined forms of the disease are also possible, for example, thrombocytopenic purpura in combination with hemorrhagic vasculitis, Fisher-Evans syndrome, characterized by a combination of immune thrombocytopenic purpura with hemolytic autoimmune anemia and nephropathy, Aldrich's syndrome with a typical triad (thrombocytopenia, otitis media, eczema), Kazabach-Merritt syndrome with thrombocytopenia, hemangiomatosis and anemia, etc.

The prognosis of thrombocytopenic purpura must always be cautious. With symptomatic thrombocytopenic purpura, the prognosis depends on the underlying process. Lethal outcomes are possible with "idiopathic" thrombocytopenic purpura (2-10%); higher mortality in newborns due to a tendency to cerebral hemorrhage.

In the treatment of thrombocytopenic purpura in children, the same therapeutic and surgical methods are used as in adults. A diet with meat restriction is prescribed, but with a large amount of fruits, vegetables, cottage cheese, liver, egg yolks, vitamins C, P, K and complex B. Fresh blood transfusions in silicate dishes, plasma, platelet and erythrocyte (with anemia) mass are recommended, epsilon-aminocaproic acid. With uterine bleeding in girls - stipticin, mammophysin. The effectiveness of glucocorticoids (prednisolone, triamcinolone, dexamethasone) in thrombocytopenic purpura in children is confirmed by most researchers, but long-term results are less encouraging than the immediate effect. Hormones are prescribed in medium doses (1 mg per 1 kg of body weight per day), by the course method, sometimes leaving a maintenance dose. With symptomatic purpura, the use of hormones is more successful.

In the absence of effectiveness from steroid therapy (over 3 months) and the duration of the disease for more than 6-12 months. splenectomy is advisable. Splenectomy is not indicated for amegakaryocytic thrombocytopenia. Early splenectomy in acute thrombocytopenic purpura is performed only with threatening bleeding and ineffectiveness of conservative therapy. Splenectomy should not be performed on newborns and young children. If the effect of splenectomy is insufficient (1/3 of cases), hormonal therapy may be more effective than before surgery. Newborns suffering from thrombocytopenic purpura are recommended exchange transfusion; corticosteroids are not effective enough.

Prevention of "idiopathic" thrombocytopenic purpura is unknown.

Skin-type diseases affect children and adults with the same frequency. One of these ailments is thrombocytopenic purpura. A photo will be discussed in the article. The disease is due to a complex nature and a special course. Consider how this process goes 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, the photo of which is presented below, is one of the varieties of the hemorrhagic phenomenon - diathesis. It is characterized by a deficiency of red blood platelets - platelets, which is often caused by the influence of immune mechanisms. Arbitrary effusions of blood into the skin act as signs of the disease, while they can be multiple or single. Also, the disease is accompanied by an increase in uterine, nasal and other blood flows. If there is a suspicion of the progression 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 shortage of platelets in the blood, and there is a tendency to increased bleeding and the development of a hemorrhagic symptom. In the presence of an ailment in the peripheral blood system, a decrease in the level of blood platelets is observed. Among all hemorrhagic types of diathesis, it is purpura that occurs most often, mainly in children of the preschool age factor. In adults and adolescents, the pathological syndrome is less common and affects mainly women.

The course of 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 the period of 6 months and progresses in adults. The recurrent form at the same time has a cyclic course, with repetitive functions of individual episodes.

Causes of the disease

In 45% of situations, the disease develops on a spontaneous basis. 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. Usually, several diseases can be distinguished among these factors.

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

Often, the manifestation of the disease occurs as part of active or passive immunization. A few more factors that provoke the formation of an ailment are taking medications, X-ray exposure, exposure to chemicals, surgical interventions, insolation.

Symptoms of thrombocytopenic purpura

The onset of any disease and manifestation is particularly acute. The sudden appearance of hemorrhages aggravates the situation and creates the appearance of the cover characteristic of the disease. Against the background of primary symptoms, there are additional signs- nosebleeds, deterioration of the general condition, pallor of the skin, a sharp increase in body temperature. If this is an acute process, a full recovery without relapse usually occurs after 1 month. Against the background of the manifestation of the disease, many children are faced with a noticeable increase in lymph nodes. Due to the mild course in about 50% of children, the disease cannot be diagnosed.

If we are talking about the chronic form of the disease, the acute onset is not provided. First symptoms appear long before acute pronounced symptoms begin. Bleeding occurs in the area of ​​​​the skin and looks like petechial hemorrhages. They exacerbate the overall picture with an exacerbation of the disease. In the first place among the formations is skin purpura - or an adult. Hemorrhagic manifestations usually appear abruptly, mainly at night.

In rare situations, "bloody tears" can be identified as a symptom, which is due to heavy bleeding from the eye area. Usually this phenomenon is observed together with skin formations. The blood goes stubbornly and abundantly, against the background of this phenomenon, anemia may occur. Gastrointestinal bleeding is less common, but in girls, the process may be accompanied by uterine pathologies and discharge. Rarely, purpura may present with hematomas and prolonged bleeding. Children suffering from an illness usually have no complaints, except that a couple of signs can be distinguished.

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

Chronic forms of the disease usually occur in children after 7 years. The course is undulating in nature, when remissions and exacerbations alternate.

Thrombocytopenic purpura in children photo

Thrombocytopenic purpura, the photo of which is presented in the article, has several forms and manifestations. The main signs of its presence in young children are hemorrhages and outpourings in the area of ​​the mucous membranes and under the skin. The color of hemorrhages varies from red to green, and the size that the body has also starts from small to large blisters and spots.

The manifestation is characterized by the absence of symmetry and logic of the sequence of formation. In children, education does not hurt and does not bother. When the stage of remission of the clinical type sets in, the disease disappears and disappears completely.

Cutaneous hemorrhagic syndrome has several specific and characteristic signs of manifestation.

• The hemorrhagic symptom in severity does not correspond to the traumatic effect. Formations can appear abruptly and unexpectedly.
• During the course of a childhood illness, polymorphism is observed, in which the variety and size indicators of rashes are different and multifaceted.
• Cutaneous hemorrhage has no favorite places of localization. For example, the body, face, and other parts of the body may appear.

The most dangerous manifestation of a childhood illness is the appearance of a hemorrhage in the sclera, which can give rise to the appearance of 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 therapeutic measures taken. Speaking about the prevalence of the disease, it can be noted that its frequency ranges from 10 to 125 cases per million population per year.

Thrombocytopenic purpura in children treatment

Before assigning effective methods of treatment, a study of the disease is carried out - a detailed diagnosis. It is possible that a decrease in the number of platelets and the greatest bleeding time will be found in the blood. There is not always a correspondence between the duration of this phenomenon and the degree of thrombocytopenia. As secondary indicators of the fact 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 this case, metaplasia in the bone marrow is manifested.
• SLE - for 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, the photo of which is presented in the article, suggests no treatment if the forms of the disease are not severe. With a moderate course, therapeutic interventions are indicated if there is an increased risk of bleeding. Such phenomena are predominantly characteristic of peptic ulcers, hypertension, duodenal ulcers. If the level of platelets in the analysis reaches the value<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 2 mg / kg of body weight per day by mouth, immunosuppressants are used (cyclophosphamide, vincristine, if other methods are ineffective). All funds are specifically prescribed by a doctor.
2. To eliminate the symptoms and features of the manifestation of the disease, human Ig is used, while the child is prescribed a dosage of 1 g per kg of body weight for two days.
3. Often used danazol, which has an androgenic effect, as well as drugs of the interferon group - ROFERON-A, REAFERON, ANTI-A.
4. Means of the symptomatic group - aminocaproic acid, etamsylate.

Forecast factors

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

Thrombocytopenic purpura in adults photo

Along with other symptoms, there is also a place to be. 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 in children and adults has many similarities, and all therapeutic methods are determined by how difficult it is and how 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, an important part of recovery is bed rest.

Medical treatment

Previously, remedies for the treatment of an ailment 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 hormonal drugs in the diet that help reduce vascular permeability and increase blood clotting. If there is multiple, as well as other symptoms, the use of several groups of drugs is effective.

1. PREDNISOLONE 2 mg per kg of body weight per day is used, in the subsequent time the dosage is reduced. Usually, this therapy gives a tremendous effect, in patients after the abolition of hormones, a relapse occurs.
2. If the measures taken in adults have not yielded results, they are used immunosuppressants. it imuran, 2-3 mg per kg of body weight per day. The duration of therapy is up to 5 months. Also winkry-steen 1-2 mg once a week.

The effect of such treatment can be detected after a few weeks, over time, the dosage is reduced. Patients should also consume a vitamin complex, especially groups P, 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 are effective (nettle, wild rose, pepper, St. John's wort).

If conservative methods of therapy have not been particularly effective, it comes to resorting to the removal of the spleen by surgical methods. 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, suggesting the possibility of quickly getting rid of the disease, is the observance of a special diet. The nutrition of the patient should be moderately high-calorie 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 use the right products.

Food to lower platelets

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

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

What to exclude from the diet

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

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