Idiopathic thrombocytopenic purpura in children. Idiopathic thrombocytopenic purpura or Werlhof's disease in children and adults Platelet purpura in children

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, general blood count indicators, coagulograms, ELISA, microscopy of blood smears, and bone marrow puncture are assessed. For therapeutic purposes, patients are prescribed corticosteroids, hemostatic drugs, cytostatic therapy, and splenectomy is performed.

General information

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

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

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

Causes of thrombocytopenic purpura

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

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

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

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

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

Symptoms of thrombocytopenic purpura

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

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

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

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

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

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

Diagnosis of thrombocytopenic purpura

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

Thrombocytopenic purpura is indicated by a sharp decrease in the number of platelets in the blood (9/l), an increase in bleeding time (>30 minutes), prothrombin time and aPTT, a decrease in the degree or absence of clot retraction. The white blood cell count is usually within normal limits; anemia occurs with significant blood loss. At the height of the hemorrhagic crisis, positive endothelial tests (pinch, tourniquet, prick) are detected. A blood smear reveals an increase in size and a decrease in granularity of platelets. Red bone marrow preparations show normal or increased amount megakaryocytes, the presence of immature forms, lacing of platelets in few points. The autoimmune nature of purpura is confirmed by the presence of antiplatelet antibodies in the blood.

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

Treatment and prognosis of thrombocytopenic purpura

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

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

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

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

THROMBOCYTOPENIC PURPURA IN CHILDREN

Thrombocytopenia (TP) - a decrease in the number of platelets per unit volume of blood, due to their increased destruction, increased consumption or insufficient formation in the bone marrow (BM).

The most common cause of TP is increased platelet destruction, while the largest share in the group of TP associated with the destruction of platelets is immune TP: autoimmune (idiopathic thrombocytopenic purpura), alloimmune (with the production of maternal antibodies against fetal antigens), isoimmune (with the production of maternal antibodies with Glanzmann's thromasthenia against fetal platelet antigens).

The most common and well-known immune TP is idiopathic thrombocytopenic purpura (AND SO ON). According to the modern definition, ITP (primary autoimmune ITP, Werlhof's disease) - autoimmune disease, characterized by isolated AFL (less than 150 thousand in 1 μl) without pronounced anemia and leukopenia, with normal or increased number CM megakaryocytes (with the exception of severe forms with a decrease in the number of megakaryocytes), the presence of antiplatelet antibodies on the surface of platelets and in the blood serum of patients, causing increased destruction of platelets, with their premature destruction by the macrophage-monocyte system. The disease is manifested by cutaneous hemorrhagic syndrome (petechiae, purpura, ecchymosis) and various bleeding from the mucous membranes. Code D 69.3 (ICD-10).

Prevalence . ITP is the most common (40%) cause of hemorrhagic syndrome in hematological practice, the most common hemorrhagic diathesis.

The incidence of new cases is from 10 to 125 per 1 million adults and children (3-8 per 100,000 children per year). The predominant age of the lesion is up to 14 years. Acute ITP predominates in children under 10 years of age (boys and girls get sick equally often), chronic ITP predominates in adolescent children (2-3 times predominant in girls).

Etiopathogenesis . According to modern concepts, ITP is a hereditary disease in which the qualitative inferiority of platelets is transmitted in an autosomal dominant manner, and plays a major role in the pathogenesis disruption of immunological tolerance to platelet self-antigens (AGs).

In 1951, Harrington first proved the presence of a circulating thrombocytopenic complex by transfusing himself with plasma from a patient with ITP, and the recipient's platelet count decreased.

Immunological tolerance is the absence of the body’s immune response to the body’s own antigens; it is established in the embryonic period and makes it possible to distinguish “self” from “foreign.”

The main link in the recognition system is the presence of the major histocompatibility complex (MHC-HLA complex) on all nucleated cells of the body; it is its molecules that present endogenous autoantigens to T-lymphocytes that recognize foreign cells.

Violation of the recognition process triggers a cascade of reactions leading to the destruction of cells with “foreign antigen” by antibodies (AT) of B lymphocytes.

Autoimmunization factors that lead to disruption of the immune system are caused to one’s own platelets: infections (viral, less often bacterial), preventive vaccinations, mental and physical injuries, hypothermia, intoxication, radiation, medications.

In modern conditions, the most common provocateurs of ITP in children are viruses (measles, parotitis, rubella, hepatitis A, HIV): they can directly affect BM (with the destruction of megakaryocytes) and cause an autoimmune response due to molecular mimicry between viruses and platelets (similarity of the platelet histocompatibility complex sites and the genome of viruses) with the production of antibodies against both viruses and viruses platelets.

In ITP it develops immune reaction hypersensitivity cytotoxic (II) type: it plays an important role in the immune system when cells foreign to the body (microbes, protozoa, worms, tumors, aging) act as antigens and becomes pathogenic when normal cells of the body (platelets) acquire autoantigenic properties.

A damaging factor (virus, medication) when connected to the platelet membrane gives it the properties of an autoantigen, triggering B-dependent immune reaction and the production of autoantibodies (immunoglobulins G or M). In addition, it is known that the alpha granules of platelets contain immunoglobulins G - in ITP, the content of the latter on the surface of the platelet sharply exceeds its internal content, and is also 200 times higher than that on the surface of the platelets of a healthy person. Immunoglobulin G is the main antiplatelet antibody (92%). In acute ITP, platelet surfaces also contain immunoglobulin M, which is associated with a previous viral infection. On the background successful treatment the content of immunoglobulin G on the surface of platelets decreases.

Antiplatelet autoantibodies in ITP are directed to various glycoproteins (GPs) of the platelet membrane: GP V (usually such children develop spontaneous remission), GP Ib, GP IIb - IIIa (high titer is typical for chronic ITP).

In the early stages of ITP development, platelets coated with autoantibodies are destroyed by the system of phagocytic mononuclear cells of the spleen, liver, and bone marrow. Next, fragments of destroyed platelets interact with free autoantibodies and form circulating immunocomplexes (CIC), in turn, deposited on the membranes of new platelets, activating complement and causing their destruction by RES cells. Thus, it develops immunological chain reaction with a decrease in platelet lifespan.

In addition to thrombocytopenia, ITP also develops thrombocytopathy – antiplatelet antibodies interact with platelet membrane receptors, which are responsible for the interaction of the latter with each other, the matrix of the vascular wall and humoral activators of platelet genesis – all this leads to disruption of platelet adhesion and aggregation.

Role of complement: if macrophages are activated through the C3B fraction of complement and attach to the platelet membrane, they are phagocytosed in RES cells, but if the C3B fraction of complement directly interacts with the platelet membrane without the participation of a macrophage, its intravascular destruction occurs, characteristic of a very severe course of ITP.

Role of the spleen: in response to the formation of an autoantigen, the spleen begins to produce autoantibodies (immunoglobulins G and M), 5 times higher than in a healthy person. In addition, the spleen is the site of destruction of platelets loaded with autoantibodies (along with the liver and bone marrow) with premature death of the latter (with ITP, the life expectancy of platelets is 8-24 hours, the norm is 6-10 days).

Bone marrow condition: in response to LT, the synthesis of poetins increases with increased thrombocytopoiesis, however, the rapid release of platelets and their release into the blood does not compensate for their rapid death under the influence of autoantibodies, despite megakaryocyte hyperplasia. In severe ITP, the process of formation and release of platelets is disrupted, since antiplatelet antibodies, with an increased number of them, can react with the membranes of megakaryocytes and destroy the latter.

Condition of the vascular wall: due to a violation of the angiotrophic function of “breadwinner” platelets and a decrease in the content of platelet-derived growth factor (stimulates DNA synthesis and endothelial proliferation), endothelial degeneration occurs. In addition, due to the commonality of the antigenic structures of platelets and endothelium, antiplatelet antibodies also destroy the endothelium, which intensifies the hemorrhagic syndrome.

State of plasma hemostasis: suffers secondarily due to a decrease in the rate of formation of active thromboplastin and increased fibrinolysis.

According to modern ideas, we can assume the following concept for the development of ITP:

Hereditary functional deficiency of platelets (defect GP IIb/IIIa, GP Ib/IX, GP V, GMP –140)

Classification . By flow distinguish between acute (less than 6 months) and chronic (more than 6 months) - with frequent relapses, rare relapses or continuously relapsing.

By gravity There are mild (“dry” purpura) forms with isolated skin manifestations and moderate and severe forms (“wet” purpura) with the addition of bleeding from the mucous membranes.

By period of illness a period of crisis (exacerbation), clinical remission (no bleeding with persistent thrombocytopenia) and clinical-hematological remission are distinguished.

Among complications ITPs include uterine bleeding, posthemorrhagic anemia, posthemorrhagic encephalopathy, etc.

Clinical picture . Acute course of ITP more common in children. The peak incidence occurs between the ages of 2 and 8 years. Boys and girls get sick equally often, in puberty girls get sick 2 times more often. Seasonality – spring.

Typically, 2-4 weeks before the onset of ITP, children suffer from ARVI or are vaccinated (80-90% of cases), after which hemorrhagic syndrome suddenly develops (after a latent period of formation of an autoimmune response).

Expressiveness clinical manifestations determined by the degree of TP: hemorrhages appear when the platelet level is less than 100 thousand in 1 μl, bleeding is less than 50 thousand in 1 μl, the threat of serious bleeding is less than 30 thousand in 1 μl.

The onset is acute, with hemorrhagic syndrome (hemorrhagic rash on the skin and mucous membranes + bleeding), an increase in body temperature to low-grade levels is possible. The patient's condition often does not change significantly.

The hemorrhagic rash in ITP is petechial-ecchymotic (bruised); in severe cases, bruising is spread throughout the body. Mainly, hemorrhages are observed on the skin over places where pressure is created (pelvic area and chest) and in areas of greatest trauma (lower legs, ankles, elbows). Small petechiae appear in places of least skin tension (supraclavicular fossa). The most favorite location for the rash is the buttocks, inner surface hips, chest, face.

Distinctive features of the rash in ITP:

1. Polychrome– hemorrhages do not appear simultaneously, there are elements on different stages development - from reddish-bluish to green and yellow.
2. Polymorphism– along with ecchymoses of varying sizes, there are petechiae, the skin becomes as if spotted, like “ leopard skins».
3. Asymmetry.
4. Spontaneity of occurrence(mainly at night) - at night the blood flow slows down (under the influence of the vagus), red blood cells in large quantities diapedically exit through the porotic wall into the surrounding tissue and in the morning a fresh bruise is detected.

Bleeding in ITP is represented in most cases by rare and short-term nasal and gastrointestinal bleeding. Some patients also experience repeated nasal and gastrointestinal bleeding (with melena), from the oral cavity (gums), kidney (gross hematuria), “bloody” tears, uterine bleeding (or profuse menorrhagia during menstruation in girls of puberty).

In 1% of patients with TP less than 20 thousand in 1 μl, fatal intracranial hemorrhages are possible within 1 month from the onset of the disease.

In 10% of cases there is moderate splenomegaly (+1-2 cm). Given the previous ARVI, slight painful lymphadenopathy of the cervical nodes is possible.

The outcome of acute ITP in 75% of cases is normalization of the platelet count within 1-2 weeks from the onset of the disease without specific therapy, in 50-65% - after 4 weeks.

Chronic course of ITP is registered when TP is less than 150 thousand in 1 μl for 6 or more months. This variant of the disease begins, as a rule, gradually, has no connection with viral infection and other provocateurs (acute onset is recorded only in 10-22% of cases of this course).

The criteria for a chronic course are: duration of more than 2-4 weeks before diagnosis, TP less than 50 thousand in 1 μl, gender (girl), age over 10 years.

The outcome of chronic ITP in 10-30% of cases is spontaneous remission several months or years after diagnosis. The incidence of complications (intracranial hemorrhage) increases to 3.3-5%.

Clinical and laboratory diagnostics . Complete blood count: posthemorrhagic anemia, thrombocytopenia I (the norm is 150-400 thousand platelets in 1 μl).

Increased bleeding time according to Duke. The tip of the finger or earlobe is pierced with a scarifier to a depth of 3 mm, spontaneously protruding blood is removed every 30 seconds by touching a paper circle (the norm is 2-4 minutes). With ITP, there is no formation of a white (platelet) thrombus, the ability of the vascular wall to contract is lost (the vasoconstrictor serotonin is not released from platelets), and the duration of bleeding is prolonged.

No or slow blood clot retraction. This indicator reflects the number and activity of platelets, since retraction (compression) occurs under the influence of an enzyme secreted by platelets (retractozyme). 3-5 ml venous blood pour into a graduated test tube and place in a thermostat for a day at T° 37°, then the whey separated from the clot is drained. By dividing the volume of serum by the volume of blood taken, the retraction index is obtained (the norm is 0.3-0.5).

Tests for capillary resistance are positive: Konchalovsky-Rumpel-Leede test (after applying a tourniquet on the skin distal to the application site, petechiae normally appear after 3 minutes, with ITP - much earlier), pinch symptom (a hemorrhagic spot appears at the site of the pinch, it gradually enlarges and becomes intense, the symptom is determined and in the stage of clinical remission), hammer sign (bruises on the skin after tapping with a percussion hammer).

General urine analysis: hematuria with renal bleeding.

Immunogram: increase in the content of the Central Election Commission.

Myelogram: megakaryocyte hyperplasia, in severe cases - a decrease in their number.

Additional research methods for ITP include determination of the level of platelet-associated antibodies (immunoglobulins G on platelet membranes), biochemical tests, platelet aggregogram (detection of thrombocytopathy), chest radiography (detection of hemorrhages), ultrasound of the abdominal organs (detection of splenomegaly), coagulogram, determination of complement and serum immunoglobulins.

Differential diagnosis . Persistent hyperplasia of the megakaryocyte lineage, changes in the myeloid and erythrocyte lineages of the bone marrow in the absence of posthemorrhagic anemia are not typical for ITP and require a search for other causes of thrombocytopenia.

Infectious diseases, in addition to thrombocytopenia, are characterized by fever, increased lymph nodes, splenomegaly, leukocytosis with neutrophilic shift, increased ESR.

Secondary symptomatic AFL for collagenosis, lymphocytic leukemia, taking medications. In leukemia, the number of megakaryocytes and platelet release are reduced; in ITP, the number of megakaryocytes decreases only in severe cases.

Hereditary hypoplastic TP with amegakaryocytosis or megakaryocytic hypoplasia - appears in the first days of life, combined with developmental defects (radial bone aplasia).

Hereditary thrombocytolytic purpuras– Wiskott-Aldrich syndrome (microcytic TP with a cell diameter of less than 2 microns, with recurrent purulent eczema), Bernard-Soulier syndrome (hemorrhagic thrombocytodystrophy, macrocytic TP, with low adhesion and aggregation, purpura and bleeding from the first months of life), May- syndrome Hegglina (macrocytic TP, manifests itself in preschool and school age, bleeding is not severe).

Congenital isoimmune TP– occurs when the fetus has platelet hypertension PLA I (2-5% in the population), which leads to sensitization of the mother, the synthesis in her body of antiplatelet antibodies, which penetrate the placenta and cause lysis of fetal platelets. Petechiae and hemorrhages appear in the first hours of life. Diagnosed by a positive thromboagglutination reaction of the child's platelets in the mother's blood serum.

Congenital transimmune TP- occurs in 30-50% of children from mothers with ITP (in half of the cases - with hemorrhagic disorders), when antiplatelet antibodies and sensitized lymphocytes penetrate from mother to fetus with the development of fetal TP.

In 1-3% of cases, a transition to ITP is registered. It is diagnosed by the detection of antiplatelet autoantibodies in the mother, and platelet lymphocytes sensitized to antigen in the mother and child.

Heteroimmune purpuras– in these cases, antibodies are produced against foreign antigens fixed on the surface of platelets (drugs such as digitoxin, PAS, sulfonamides, hypothiazide, as well as rubella viruses, chickenpox, measles), when after 2-3 days from the start of treatment or a viral infection, petechiae and hemorrhages occur, but after 2-3 weeks there is a complete recovery (when it becomes chronic, a diagnosis of ITP is made).

Outcomes . In most patients, self-healing occurs within 1-6 months (in children - in 10-30% of cases). Risk factors for the development of chronic ITP are: female gender, age over 10 years, severe bleeding at the onset of the disease. The mortality rate for ITP is less than 1% (caused by hemorrhages in the central nervous system, severe posthemorrhagic anemia).

Principles of treatment . Basic principles of treatment, based on the destruction of platelets loaded with autoantibodies by RES cells, are:

1. Decreased production of autoantibodies.
2. Impaired binding of autoantibodies to platelets.
3. Elimination of destruction of platelets loaded with autoantibodies by RES cells.

Indications for hospitalization:

1. Severe, life-threatening bleeding, regardless of platelet count.
2. TP less than 20 thousand in 1 µl.
3. Necessity inpatient treatment(tamponade).
4. Accommodation away from the hospital or at the request of parents.

Indications for outpatient treatment:

1. TP 20-30 thousand in 1 µl in the absence of hemorrhagic syndrome.
2. TP more than 30 thousand in 1 μl in the absence or minimal manifestations of hemorrhagic syndrome.

Taking into account the fact that 30-70% of children with severe ITP reach a platelet level of 50-100 thousand in 1 μl after 3 weeks only on symptomatic therapy, an individual approach to the treatment of each patient is required.

In the acute period it is prescribed BED REST, then the regime is gradually expanded. DIET with the exclusion of obligate allergens.

DRUGS THAT IMPROVE THE ADHESIVE-AGGREGATION FUNCTION OF PLATELETS, are indicated for any form of ITP (if DIC syndrome is excluded):

Epsilon-aminocaproic acid– powders, granules in a bottle (when diluted to the 100 ml mark with water, a 20% solution is obtained – 0.2 g in 1 ml). Single dose (SD) with moderate fibrinolytic activity for children under 1 year 2.5 ml (0.5 g), 1-7 years 2.5-5 ml (0.5-1 g), 7-10 years 15 ml (3 g). RD at acute bleeding in children under 1 year 5 ml (1 g), 1-4 years 5.0-7.5 ml (1-1.5 g), 4-8 years 7.5-10 ml (1.5-2 g) ), 8-10 years 15 ml (3 g). In emergency cases, it is prescribed intravenously in the form of a 5% solution of 0.9% sodium chloride solution at a dose of 1 ml/kg at a rate of 20-30 drops per minute, repeated drip infusion if bleeding continues - after 6-8 hours.

Recombinant activated factorVII(eptagon-alpha) is the most effective hemostatic drug that allows you to correct massive diffuse hemorrhages in ITP and platelet dysfunctions refractory to transfusions of blood components. In case of severe hemorrhagic complications, it is preferable to use the drug as early as possible from the onset of bleeding, at a dose of 90 mcg/kg.

Carbazochrome(adroxon) – ampoules of 0.025% solution, 1.0 ml each. It is prescribed subcutaneously or intramuscularly at 0.5-1.0 ml 1-4 times a day.

Etamzilat(dicinone) - tablets of 0.25 (¼-1 tablet every 4-6 hours), ampoules of 12.5% ​​solution of 2.0 ml (2-4 ml intravenously or intramuscularly in surgery for the prevention and treatment of postoperative bleeding).

Calcium pantothenate(vitamin B 5) – tablets of 0.1. RD in children 1-3 years old 0.05-0.1, 3-14 years old 0.1-0.2 2 times a day, after meals.

Magnesium preparations orally: basic magnesium carbonate in powders, RD in children under 1 year 0.5, 2-5 years 1-1.5, 6-12 years 1-2 g 2-3 times a day.

Recombinant activated factor VII of blood coagulation (drug NovoSeven, Novo Nordisk, Denmark) has proven effective in the treatment and prevention of hemorrhagic syndrome caused by a decrease in the number or dysfunction of platelets. The use of the drug is under study, according to M.B. Belogurova et al (2004) for hemorrhagic syndrome due to ITP NovoSeven is used at a dose of 100 mcg/kg 2 times (acute ITP) or 70-120 mcg/kg once (chronic AND SO ON).

VASCULAR STRENGTHENING DRUGS also indicated for any form of ITP.

Ascorbic acid- in powders. RD in children under 1 year 50 mg, 1-7 years 75-100 mg, 7-14 years 100-200 mg 3 times a day.

Rutin(vitamin P) – tablets 0.2, powders. RD in children under 1 year 5-10 mg, 1-2 years 10 mg, 3-5 years 10-15 mg, 6-8 years 15-20 mg, 9-15 years 20-40 mg.

Combination of ascorbic acid and rutin, ascorutin(ascorbic acid 0.1 or 0.2 + rutin 0.02 or 0.01) in tablets.

Calcium chloride 5% solution for children under 1 year, 10% - over 1 year, prescribed 1 teaspoon - 1 tablespoon 3 times a day after meals.

Calcium gluconate– powders, tablets 0.5. Daily dose (DD) for children under 1 year 0.5, 2-4 years 1.0, 5-6 years 1-1.5, 7-9 years 1.5-2, 10-14 years 2-3 g 3 times a day, before meals.

ANTIHISTAMINES are prescribed in courses of 7-10 days, taking into account the immunopathological genesis of ITP.

MODERN SPECIFIC THERAPY FOR ITP includes the use of glucocorticoids (GC), intravenous immunoglobulin (IVIG), anti-Rhesus D-immunoglobulin, alpha-2 interferon drugs, surgical removal of the spleen (splenectomy).

GLUCOCORTICOIDS have anti-inflammatory, desensitizing, antiallergic activity, and immunosuppressive effects. Before the introduction of RES cell blockers (IVIG, anti-Rh-D immunoglobulin), GCs were the only therapeutic agent for the treatment of patients with acute ITP and are still the basic maintenance therapy for patients with chronic ITP. Indications for the use of GCs are generalized cutaneous hemorrhagic syndrome in combination with bleeding of the mucous membranes, hemorrhages in the sclera and retina, “wet” purpura with posthemorrhagic anemia, hemorrhages in internal organs.

For ITP, three modes of GC administration are most often used:

1. Standard doses of oral GCs: prednisolone 1-2 mg/kg per day or 60 mg per m2. within 21 days.
2. High doses of oral GCs: prednisolone 4-8 mg/kg per day for 7 days or methylprednisolone 10-30 mg/kg per day for 3-7 days with rapid discontinuation of the drug.
3. High doses of parenteral GCs: methylprednisolone 10-30 mg/kg per day for 3-7 days (“pulse therapy”).

The latter regimen is most effective in children - the platelet count rises to more than 50 thousand/μl on the 4th day (on the 16th day in those untreated or receiving standard doses of GC).

In chronic ITP, achieving a complete response (more than 100 thousand/μl) is possible in 30% of patients, a partial response (less than 40 thousand/μl) is possible in 15-20%, and in 50% of cases there is no response. Complete long-term remission was observed only in 10-20% of cases.

For steroid-resistant ITP, pulse therapy with dexamethasone is performed at a dose of 0.5 mg/kg per day (not more than 40 mg) for 4 days every 28 days of oral administration, for a total of 6 cycles.

Human immunoglobulin normal for intravenous administration, INTRAVENOUS IMMUNOGLOBULIN(IVIG) is a preparation of normal polyspecific immunoglobulin G, obtained from a pool of sera of several thousand donors, with the same distribution into subclasses as in normal serum and a half-life of about 3 weeks (for example, sandoglobulin from Sandos, Octagam from Octapharma ).

The mechanism of action of IVIG is a reversible blockade of Fc receptors of RES cells, which prevents the removal from the bloodstream of circulating platelets sensitized by autoantibodies (immunoglobulins G), reversible blockade of Fc receptors of leukocytes, Fc-dependent reverse inhibition of the synthesis of autoantibodies by B lymphocytes, modulation of suppressor and helper T-lymphocyte activity, suppression of complement-dependent tissue damage. Blockade of Fc receptors of RES cells explains the rapid increase in platelets within several hours after IVIG administration.

IVIG is prescribed at a dose of 1000 mg/kg once or 2000 mg/kg for 2-5 days with equal effectiveness (in 83% of cases there is an increase in platelets above 50 thousand/μl, in 64% above 100 thousand/μl). If in the first 48 hours of therapy the platelet count increases by less than 30 thousand/μl, further therapy is useless.

In children with acute ITP, complete hematological remission over a long period of time is achieved in 62-68% of cases. In chronic ITP, the effect is less pronounced (transient in 50%, complete and partial in 25%), however, repeated infusions make it possible to avoid splenectomy in 50-67% of children, which is especially important when the age is less than 6-10 years.

The main disadvantages of IVIG treatment are the high cost ($5,000-10,000 per course for a 10-year-old child) and the transient effect in chronic ITP.

Anti-D (Rh) immunoglobulin,ANTI-RHESUS-D-IMMUNOGLOBULIN– sterile freeze-dried fraction of immunoglobulin G containing antibodies to Rhesus (D). The mechanism of action is similar to IVIG - the Fc receptors of the RES cells are blocked by anti-erythrocyte antibodies - isohemagglutinins.

The drug is available in a dosage of 15,000 IU (300 μg of anti-Rhesus D-immunoglobulin), 1250 IU (Rezonativ, Octapharma), administered to Rh-positive patients intramuscularly and intravenously. Due to the risk of developing hemolytic anemia, the dose depends on the concentration of Hb: 25-40 mcg/kg with Hb less than 100 g/l, 40-60 mcg/kg with Hb more than 100 g/l (optimal dose 50 mcg/kg). Perhaps a single intravenous injection or 2-5 daily intramuscular injections. The drug is effective in 79-90% (an increase in platelets is noted on days 2-8 of treatment, the duration of response is on average 5 weeks). This drug is not used for life-threatening conditions (it slowly increases platelet levels).

The cost of anti-Rh-D immunoglobulin is 1/10 of IVIG. The drug is used as long-term maintenance therapy in patients with chronic ITP and allows delaying splenectomy. A side effect may be hemolytic anemia (transient, 3-7 days after administration, lasts 1-2 weeks, Hb decreases by 5-20 g/l).

ALPHA-2-INTERFERON– genetically engineered drug (Reaferon, Intron A, Reaferon-A), administered subcutaneously or intramuscularly 3 times a week for 3 months. For children under 5 years of age, the dose of the drug is 500,000 IU/day, 5-12 years old 1,000,000 IU/day, over 12 years old 2,000,000 IU/day. The effectiveness is 72.5%; in most patients, the hematological effect is observed after 2 weeks. The duration of therapy should be at least 4 weeks. If the platelet count increases during the first 4 weeks, therapy at the same dose continues for up to 9 weeks, then continues as maintenance therapy.

PLATELET TRANSFUSION for ITP they are not effective; they are performed only for life-threatening bleeding. The number of platelets in the peripheral blood does not increase, but a local hemostatic effect is possible.

SPLENECTOMY indicated in the absence of effect from conservative therapy, the presence of complete deep TP, hemorrhagic syndrome with life-threatening bleeding.

Hematological indications for splenectomy are: 1. Duration of ITP for more than 12 months. 2. TP less than 10 thousand/µl with a history of bleeding. 3. TP 10-30 thousand/µl with severe bleeding.

In 72-80% of cases after surgery, complete hematological remission is achieved, in 15% a relapse occurs due to the presence of additional spleens, which is confirmed by the absence of erythrocytes with Hovel-Joly bodies in the blood of operated patients (they necessarily appear when complete removal spleen). In this case, an additional splenectomy is performed.

If splenectomy is ineffective, glucocorticoids are again prescribed for a short time, and if this does not lead to an increase in the number of platelets, CYTOSTATICS: vincristine 1.5 mg/m2 intravenously once a week. The effect occurs after 2-4 injections (longer administration is not advisable).

In addition to cytostatics, after splenectomy it is possible to use SYNTHETIC ANDROGENS: danazol at a dose of 20 mg/kg per day (not more than 800 mg, divided into 2 doses) for 1-12 months.

An alternative to splenectomy is X-RAY ENDOVASCULAR OCCLUSION OF THE SPLEN (REO)– introduction of vasopressin through a catheter into the splenic artery, causing its spasm, with the subsequent development of splenic infarction. If patients with chronic ITP do not respond to REO, splenectomy will not help. In the treatment of ITP when splenectomy is contraindicated, irradiation of the spleen is indicated, but such treatment is not recommended for children with chronic ITP.

Dispensary observation . During clinical remission, platelet levels are monitored monthly. In case of long-term clinical and hematological remission (more than 5 years after acute ITP), the child is removed from the dispensary register. In chronic ITP, the child is observed until transfer to an adult clinic.

Upon discharge from the hospital, it is recommended: observation by a hematologist at the place of residence; refusal of physical treatment, insolation; refusal of aspirin, carbenicillin; herbal medicine for the prevention of bleeding (infusions of chamomile, nettle, rosehip) in courses of 15 days every 3 months; exemption from physical education and sports; registration of disability in case of persistent chronicity of ITP.

It is necessary to follow a diet with the exception of spicy foods, vinegar, canned vegetables, and alcohol. It should also be prescribed for a long time Sesame oil, peanuts.

Vaccinations while taking antihistamines are possible only 1 year after acute ITP. Vaccinations with live viral vaccines are contraindicated.

Represents a symptom pathological condition called thrombocytopenia. It is associated with a reduced number of platelet cells in the peripheral blood.

Thrombocytopenia is manifested by various blood diseases and is accompanied by hemorrhagic blood syndrome of the petechial-bruise type, i.e. like purpura.

Classification of the disease

Considering mechanism of occurrence thrombocytopenia, purpura is classified as follows:

1. Thrombocytopenic syndromes of congenital hereditary nature: thrombocytopenia with absence of the radius, Wiskott-Aldrich syndrome, Hegglin syndrome, Bernard-Soulier syndrome.

2. Thrombocytopenia is congenital, but not hereditary. Occurs as a result of intrauterine infectious lesions fetal infection, such as rubella or chickenpox. Immune thrombocytopenia occurs in the mother with antibodies through the placenta. Alloimmune purpura forms in newborns when the children's platelets inherit antigens that the mother does not have.

3. Acquired thrombocytopenia. The disease can be presented in several forms: immune, when autoimmune idiopathic purpura and infectious disease clinics predominate (HIV infections, mycoplasma, malaria, etc.); thrombotic purpura; hypersplenism; DIC syndrome (consumption syndrome); massive transfusion syndrome; thrombocytopenia in pregnant women.

Symptoms and signs

In rare cases medical science faced with a diagnosis such as thrombotic thrombocytopenic purpura. This form of thrombocytopenia is characterized by an acute onset and a rapidly progressive course. The disease affects people young and often becomes fatal.

To date, the causes of the disease are not clear. At the initial stages of this type of purpura, platelet microthrombi are formed, spreading in small vessels (capillaries and arterioles) throughout the body, especially affecting the brain, kidneys and liver, as a result of which blood flow is disrupted, cells and tissues are damaged.

Thrombocytopenic purpura manifests itself in a complex manner and includes a number of signs: a low platelet count in the blood, the presence of hemolytic anemia, neurological changes, impaired renal function, and the appearance of a febrile state.

In the case of the acquired form, it often occurs allergic purpura or Schonlein purpura. In most cases, the circulatory system of children aged 3-7 years is affected. No specific signs of the disease have been identified to date, but ESR indicators in the blood correlate with the acute degree of vasculitis.

Pigmented chronic purpura has a polymorphic nature of symptoms, but there are symptoms characteristic exclusively of it: the presence of erythema, pigmentation and hemorrhage, the degree of which depends on the level of vascular damage. Such purpura is accompanied by itching and has a recurrent nature: each time it is localized over an increasingly larger surface, the rash acquires a brighter color.

Hemorrhagic form of purpura manifests itself as a rash similar to the rash associated with diathesis, but gradually merging into large spots. The rashes are distributed on the limbs, less often on the torso. Exactly skin rash is the only sign of the disease.

Possible complications

Most severe complication purpura is a cerebral hemorrhage and meninges, rarely - into the sclera or retina.

For purple heavy bleeding possible during abdominal surgery, tooth extraction and during childbirth.

Causes of the disease

In medicine, there are a number of factors that can provoke a decrease in the number of platelets in the blood with the subsequent appearance of microthrombi: the infectious nature of diseases, incl. HIV infection; taking certain medications; pregnancy period; diseases of an autoimmune nature in the presence of an inflammatory process; hemolytic-uremic syndromes in children, provoked by Escherichia coli and the presence of toxins.

Appearance vascular purpura associated with dysfunction of blood vessels in hemostasis. More often vascular purpura is congenital and appears as a hemangioma. Such spots appear in every tenth newborn and eventually disappear or are removed with a laser.

At congenital purpura genetic Marfan syndrome or Ekhlar-Danlos. In such cases, complex violations are identified connective tissue, as a result of which blood vessels, especially arteries, also receive defects.

Reasons allergic purpura are inflammatory processes in the capillaries, but their etiology still remains unknown. There are suggestions about the influence of environmental factors, nutrition and infectious diseases.

TO specific symptoms purpura can be attributed to the appearance of pain in the joints, inflammatory processes in the esophagus, as well as neurological symptoms.

Appearance vascular purpura common in older people. Sometimes it is also called senile pigmentation. The appearance of the disease is associated with wear and tear of blood vessels, deterioration of their functioning, especially in areas that are especially susceptible to ultraviolet radiation (limbs, face).

The rashes and spots may become more intense due to increased blood pressure. Most often, the reason lies in a sudden complication caused by a blood clot formed in a vein, sudden lifting of a heavy load, severe coughing, during the pushing period during childbirth, and even vomiting. Vascular regeneration can occur without the use of drug treatment.

Diagnosis and treatment

The diagnosis is established after receiving the results of a blood test in accordance with the following indicators:

1. The level of thrombocytopenia in the peripheral blood test is below 100.0×109/l.
2. Autoantibodies to platelets are present.
3. Signs of diseases such as anemia and acute leukemia, which are characterized by impaired hematopoietic functions.
4. You are not taking medications that can cause thrombocytopenia.
5. The content of megakaryocytes in bone marrow structures is normal. But there is one peculiarity - bone marrow puncture is possible only if the patient has reached the age of 60 or a splenectomy is required.

Treatment of purpura is primarily aimed at correcting the hemorrhagic syndrome, and only then at increasing the number of platelets in the blood. This is due to the fact that patients in most cases are resistant to low platelet counts, but the side effects of therapy can be more serious than the consequences of purpura.

Purpura, which does not cause inconvenience to the patient and is not accompanied by serious changes in the composition of the blood, does not require specific treatment. In this case, there is a need for constant monitoring by the attending physician.

If bleeding occurs, it is prescribed medications local hemostatic effect (cream, ointment), as well as hormonal agents.

An effective treatment method is blood and/or platelet transfusion.

If bleeding becomes systematic, and the use of medications does not produce results within six months, then splenectomy is prescribed. Removal of the spleen is a drastic method, which is resorted to when there is a threat of hemorrhage in the brain.

Before the operation, a medical examination is required, an examination of the abdominal and chest organs is prescribed, and a repeat blood test is given. The doctor may additionally prescribe: x-rays, ECG, magnetic resonance imaging.

Brief interesting data
- Idiopathic thrombocytopenic purpura before the age of 10 occurs with the same frequency in boys and girls, and after 10 years in adults, and many times more often in women than in men.
- Purpura most often appears in spring and autumn.
- Possible in 10% of cases death, associated not with purpura as a disease, but with its consequence - hemorrhage in the brain. This is due to the fact that patients delay the period of contacting specialists.

Immediately before the operation, the patient is vaccinated in order to prevent possible inflammatory processes after removal of the spleen.

Splenectomy is performed under general anesthesia. Either laparoscopy or abdominal surgery is performed depending on the indications and capabilities of the medical institution.

Full recovery after such an operation occurs after 1-1.5 months. After surgery, the risk of infection increases, so regular vaccination against influenza virus and other infections is required as prescribed by a doctor.

After a course of treatment or discharge from hospital, it is necessary to adhere to a certain daily routine in order to avoid recurrent manifestations.

It is important to establish a proper sleep and wakefulness regime (you should rest at least 8-10 hours a day); do daily morning exercises (about half an hour) and water treatments in the form of a warm shower; study physical exercise, avoiding overloads; set aside 1-2 hours for nap; should get into the habit of daily walks in the fresh air (at least 1.5 hours); Avoid direct sunlight.

Particular attention should be paid to the issue of nutrition: food should be taken in small portions 5-6 times a day. First of all, the diet includes proteins (fish, white meat), plant foods and dairy products.

Necessary component seasonal berries, vegetables and fruits, as well as all kinds of non-carbonated and non-alcoholic drinks (mousses, compotes, herbal teas) should be included.

The prognosis for recovery is quite favorable if a competent treatment program is developed and all the instructions of the attending physician are followed. Deaths are possible only in rare cases and are associated with the fact that patients delay the period of contacting a medical facility and bleeding in the brain occurs.

Prevention

The appearance of purpura in childhood and adolescence is the most common occurrence, so it is necessary to pay attention Special attention health of children who have suffered severe infectious disease. The most competent decision of parents should be observation by a hematologist for several years.

Preventive measures regarding relapses come down to maximum avoidance of viral infections and prevention of bacterial diseases.

It is necessary to be exposed to direct sunlight as little as possible, and it is better to completely eliminate such situations.

Besides, preventive actions consist of the maximum possible elimination foci of infection: caries, tonsillitis, etc.

Traditional methods of treatment

An effective remedy to combat purpura is the use of sesame oil. Particularly significant results can be achieved if it is used in complex hormonal therapy. The oil helps increase the number of platelets and increases blood clotting. It is enough to consume sesame oil 3 times a day 30 minutes before meals in the amount of 1 teaspoon.

For bleeding and to strengthen the immune system during purpura, take an infusion of rose hips. A glass of dried berries is poured with 1 liter of boiling water and infused for 10-12 hours (it is good to brew the infusion overnight). Drink during the day instead of tea. You can add honey or a small amount of Sahara.

For external use, a tincture of Japanese sophora is useful. The composition is prepared as follows: 1 teaspoon of fruit is poured into 100 ml of boiling water, infused for 1-1.5 hours, filtered. The affected areas are lubricated with tincture in the morning and evening.

Thrombocytopenic purpura is a disease characterized by hemorrhagic manifestations in the form of hemorrhages under the skin and bleeding resulting from a decrease in the number of platelets responsible for blood clotting. This is one of the common forms of blood diseases in children. Causes of the disease. There are primary and secondary (symptomatic) forms of thrombocytopenic purpura.

Primary forms can be either hereditary or acquired due to a previous disease; secondary forms arise as signs of a number of diseases. The mechanism of development of primary and secondary thrombocytopenic purpura may be similar (for example, due to an autoimmune process - an immunological attack - the destruction of certain structures of the body by the body's cells). Most often, the disease develops in childhood.

What is purpura

Purpura - medical symptom, characteristic of the pathology of one or more parts of hemostasis. Purpura refers to small-spotted capillary hemorrhages in the skin, under the skin or in the mucous membranes. Single hemorrhages can be pinpoint (petechiae), less often strip-like (vibex), small-spotted (ecchymoses) or large-spotted (bruises). Usually observed in the form of multiple petechiae and ecchymoses with a diameter of up to 1 cm. Purpura is nonspecific symptom, which manifests itself in a number of diseases, but is based on common development mechanisms.

Thrombocytopenic purpura is a primary hemorrhagic diathesis, in which a decrease in the number of platelets in the blood is determined with an increased or normal content of cells from which platelets are formed in the bone marrow. This is one of the most common forms of hemorrhagic diathesis. Thrombocytopenic purpura refers to those conditions with a reduced platelet count, the causes of which are unknown and which are not signs of other diseases.

Also, the factors influencing the occurrence of thrombocytopenic purpura have not been fully elucidated. The disease develops even in infants, although it most often occurs in children 3-6 years old. Before the age of 14, the disease occurs equally often in both boys and girls. However, at high school age, the incidence in girls is 2-3 times higher than in boys. Importance is attached to the infectious factor preceding the development of thrombocytopenic purpura. Most often in children, the disease begins after a viral infection, less often after a bacterial one.

It is currently believed that the decrease in platelet count in this disease is caused by increased destruction of blood platelets. When the number of destroyed blood platelets exceeds potential opportunities bone marrow to compensate for the lack of platelets in the blood, thrombocytopenia occurs. In the development of the disease when blood platelets are destroyed, immunological disorders such as a decrease in the body's defenses and the formation of a small number of protective cells play an important role.

But it is assumed that the mechanism of development of thrombocytopenic purpura involves several reactions that determine the course of the disease. The spleen also plays an important role in the development of thrombocytopenic purpura, since it is in it that increased destruction of platelets occurs due to the immunological process. At the same time, the spleen is the main site of production of antibodies that destroy platelets in autoimmune thrombocytopenic purpura, and two forms are distinguished - autoimmune and heteroimmune thrombocytopenic purpura.

In the autoimmune form, antibodies are produced in the spleen and thymus gland, subsequently causing increased destruction of platelets, which cannot be compensated by the body, in particular the bone marrow. In an autoimmune process, antibodies are produced against one's own unchanged platelets. This determines the signs of thrombocytopenic purpura, which takes a chronic course. The heteroimmune form of thrombocytopenic purpura occurs when the structure of the platelet is disrupted, i.e. when a new complex is formed, which is formed under the influence of viruses, medications or other foreign substances.

Antibodies are produced against this complex, which also leads to the destruction of platelets. This mechanism determines the signs of an acute disease. A classic example of a heteroimmune form is thrombocytopenia, which develops when taking medications. Such drugs may include quinine, salicylates, sulfonamides, antibiotics, etc.

The heteroimmune form of thrombocytopenic purpura (acute form of Werlhof's disease), which is formed with the participation of viruses and bacteria, has similar development mechanisms. In the development of bleeding in thrombocytopenic purpura, damage to the vascular wall and blood coagulation system plays an important role.

Platelets perform important functions in the body: they protect the walls of blood vessels from damage, take part in blood clotting and influence fibrinolysis - the dissolution of intravascular blood clots. Platelets are the natural feeders of blood cells and lymphatic vessels, therefore, when they are deficient, vascular permeability increases (substances easily exit and penetrate into them) and their fragility, which leads to the development of spotty hemorrhages on the skin.

A decrease in the level of serotonin in the blood in patients with thrombocytopenic purpura plays a certain role in the development of bleeding. With the help of this substance, nerve impulses are carried from organs to the brain and back. Serotonin also has vasoconstrictor effect and stimulates platelet aggregation.

The combination of these factors (thrombocytopenia, functional disorders vascular wall, decreased serotonin levels) determines the manifestation of thrombocytopenic purpura. In the development of bleeding in thrombocytopenic purpura, disturbances in the processes of blood coagulation and fibrinolysis are important.

Symptoms of thrombocytopenic purpura

Along the way, acute and chronic forms of thrombocytopenic purpura are distinguished. The acute form lasts up to 6 months and ends with recovery. Subsequently, the re-development of signs of the disease does not occur. In children, the acute form of thrombocytopenic purpura occurs more often than the chronic form, mainly in the younger age group - from 1 year to 5 years. As a rule, it is preceded by certain factors: viral infections, vaccinations, etc. The typical period between exposure to the factor and the onset of the disease is 1-3 weeks.

The onset of the disease is acute, sudden hemorrhages appear in the skin and mucous membranes, nosebleeds, deterioration general condition child, signs of bleeding (pale skin, low blood pressure), body temperature rises to 38 ° C. Duration of illness at acute form thrombocytopenic purpura, as a rule, does not exceed 1 month, during which a rapid restoration of all disorders occurs and recovery occurs.

Many children with acute course lymphadenopathy is observed - inflammation of the lymph nodes. Acute thrombocytopenic purpura may result in spontaneous recovery. In a number of children, the disease is not diagnosed, as it is mild, with minimal signs of bleeding. In the acute form, the course of the disease is favorable - complete recovery usually occurs within 1-3 months. The chronic form of thrombocytopenic purpura is not characterized by an acute onset. The first signs of the disease appear long before the characteristic manifestations of the disease and are often not noticed by either the patient or his family.

The first signs of bleeding appear on the skin in the form of pinpoint (petechial) hemorrhages, mild nosebleeds and bleeding after surgical intervention. During the period of exacerbation of thrombocytopenic purpura, various types of hemorrhages predominate. Among them, in first place in terms of frequency of occurrence are skin manifestations - purpura itself. Hemorrhages usually appear suddenly without cause or after microtrauma. Moreover, spontaneous hemorrhages are often observed at night.

With thrombocytopenic purpura, the symptom of “bloody tears”, caused by bleeding from the eyes, is very rarely observed. The second most common symptom during the course of thrombocytopenic purpura is bleeding. As a rule, bleeding is observed along with skin manifestations. Nosebleeds are the most common. Usually they are persistent, abundant, and lead to anemia.

Frequent bleeding from the mucous membranes of the oral cavity, from the tonsils and back wall throats. Less common are gastrointestinal bleeding, kidney bleeding, and in girls - uterine bleeding. Frequent or heavy bleeding with thrombocytopenic purpura leads to the development of posthemorrhagic anemia.

Thrombocytopenic purpura is not characterized by intermuscular accumulations of blood and hemarthrosis, i.e. accumulations of blood in the joint cavity, subcutaneous hematomas and prolonged bleeding when the integrity of the skin is violated. Many children suffering from thrombocytopenic purpura have no complaints. As a rule, they appear with the development of posthemorrhagic anemia.

There is lethargy, irritability, weakness, fast fatiguability. The temperature is normal. Enlarged liver and spleen are not typical for thrombocytopenic purpura. The duration of the disease for more than 6 months is considered a chronic form of the disease; most often it takes this course in children 7-10 years old. The chronic form of thrombocytopenic purpura has a protracted wave-like course, when periods of improvement are again replaced by periods of exacerbation.

Detailed descriptions of symptoms

Treatment of thrombocytopenic purpura

Treatment methods. The set of measures for thrombocytopenic purpura is determined by the severity, course and period of the disease and consists of conservative (with the help of medications) and operational methods. For any manifestations of purpura, hospitalization in specialized departments of the hospital is recommended. bed rest. The diet should be complete, high-calorie, food is given chilled, liquid, in small portions.

In the treatment of thrombocytopenic purpura with drugs, a special place is occupied by hormonal drugs that reduce vascular permeability, increase blood clotting and increase immunity. Hormones are used for extensive lesions skin, bleeding from mucous membranes, bleeding of various locations, hemorrhages in internal organs, severe thrombocytopenia. Prednisolone is most often used, initially in large doses.

The effect of treatment appears after 1-2 weeks, then the dose is gradually reduced. Patients are prescribed a complex of vitamins - it is advisable to introduce vitamins P and C. Large doses Vitamin C is not recommended, as it impairs the functional properties of platelets. Prescribe calcium salts (calcium pantothenate), aminocaproic acid orally 0.2 g/kg body weight 3-4 times a day or intravenously in the form of a 5% solution.

Dicinone has an effect on the vascular wall; it is prescribed orally or administered intramuscularly. ATP in combination with magnesium sulfate has a certain effect on the functional properties of platelets. Used as hemostatic agents medicinal collection, including nettle, rose hips, corn silk, water pepper, yarrow.

Transfusions of blood components are used only in cases of acute severe anemia that poses a threat to life, or to stop massive bleeding that cannot be eliminated by other means. If conservative therapy for thrombocytopenic purpura is ineffective, splenectomy is resorted to ( surgical removal spleen).

Cytostatic drugs that reduce cell growth and reproduction are prescribed only in extreme cases, when all available means have been used. Treatment with cytostatic drugs is usually carried out in combination with hormonal drugs. The following drugs are used:

• imuran,
• cyclophosphamide,
• 6-mercaptopurine vincristine,
• vinblastine.

Patients with thrombocytopenic purpura are subject to dispensary registration for 5 years.

Questions and answers on the topic "Thrombocytopenic purpura"

Question:Hello! Tell me what to do with two such diagnoses. I acquired varicose veins after my third birth, and refused the proposed operation, because many of my friends did not have this operation without relapses, and it was not possible after the operation to maintain the “self-protection” mode (heaviness, constantly on my feet). I supported my legs various tinctures. And yesterday they diagnosed purpura - platelets 20, prescribed medrol 32, calyposis, etamzilate. How can I not aggravate the condition of my legs, how can I support them while the blood is being restored? The doctor, the fact that I have varicose veins has not responded in any way, but my leg is going numb and cramps are emerging.

Answer: Hello! IN currently we should really be talking about maintaining the condition of the veins until the blood is restored. The most effective remedy for this is medical compression hosiery(not all knitwear that can be purchased in a pharmacy chain, and not only, is not such). Contact a phlebologist. Knitwear is selected strictly individually (from size to degree of compression). All kinds of ointments, gels, tablets and capsules - aids, I do not mean the drugs you take to treat purpura.

Question:Good afternoon I am 35 years old. Please tell me if I can carry and give birth to a child with a diagnosis of idiopathic thrombocytopenia. The disease is not congenital, but acquired at the age of 23 years. From 1999 to 2006 I took prednisolone to maintain my platelets. In 2006, I had a splenectomy and my platelet levels returned to almost normal. On this period I have no bleeding in my life. The platelet level fluctuates, but does not drop below 80-90 (and even then during menstruation). Your period runs like clockwork at a certain time, without interruption, for 2-3 days. There are no problems as a woman. Help.

Answer: In most cases, pregnancy does not worsen the condition of patients with idiopathic thrombocytopenic purpura, but in 20-30% of pregnant women an exacerbation of the disease may occur. I also recommend consultation with a therapist and hematologist.

Thrombocytopenic purpura refers to hemorrhagic diathesis that occurs with a violation of the platelet component of hemostasis, where the number of blood platelets falls below the acceptable level (150 x 10 9 / l). This phenomenon occurs under circumstances that contribute to the fact that bone marrow begins to be over-degraded, over-consumed, or under-proliferate.

Most often (TP) occurs with increased cell destruction, although all these processes are also not excluded in one patient, they are combined with each other, run in parallel and, naturally, aggravate the situation. The platelet count in such cases drops to critical levels, which in turn determines the severity of the disease.

Prerequisites for the occurrence of thrombocytopenia

Most thrombocytopenias, as has already been proven, are acquired., that is, genetically programmed defects are not the basis of the disease, although isolated cases of hereditary pathology still sometimes occur:

1. Impaired synthesis of thrombocytopoietins in the human body is associated with hereditary thrombocytopenia;
2. The deficiency of enzymes that carry out glycolysis or the Krebs cycle is also a genetic abnormality.

All other conditions characterized by a decrease in the platelet level are divided into immune and non-immune, which have their own specific causes.

Non-immune thrombocytopenia

The starting factors for the occurrence of non-immune thrombocytopenia are:

• Mechanical effects on platelets, causing their injury (vascular prosthetics, splenomegaly, giant hemangiomas);
• Tumors with metastases to the bone marrow;
• A disorder of hematopoiesis, accompanied by sluggish cell proliferation, which is characteristic of aplastic disease, which is often accompanied by;
• Exposure to radiation or chemicals that damage myelopoiesis;
• High need for platelets with a lack of folic acid or Vit B 12, disseminated platelet aggregation - DAT, RDS - respiratory distress syndrome, thrombosis, long-term therapy with low doses of heparin).

Thrombotic thrombocytopenic purpura is also classified as a non-immune variant of TP.(TTP), which has an acute onset and is characterized by a malignant course. The etiology of this disease is still unclear, but it is known that it is usually fatal. It has been noted that TTP occurs more often in adults in the following cases:

1. Previous bacterial or viral infection;
2. Vaccinations;
3. Presence of HIV infection;
4. Pregnancy;
5. Use of oral contraceptives;
6. Treatment with certain anticancer drugs;
7. Collagenoses;
8. As a hereditary pathology (very rare).

Thrombotic thrombocytopenic purpura is characterized by the deposition of hyaline platelet thrombi, the formation of which was caused by spontaneous aggregation of platelets in small-caliber vessels, as a result of which they close the vessel. Platelet blood clots invade the entire human body and damage the microvessels of many organs, so TTP is characterized by the presence of symptoms:

• Hemolytic anemia;
• Fever;
• Neurological symptoms;
• Acute renal failure.

The death of the patient usually occurs as a result of kidney failure (RF).

Production of antiplatelet antibodies is a path to immune thrombocytopenia

Immune thrombocytopenic purpura has several types:

Autoimmune thrombocytopenia also has its own division depending on the direction of antibodies and the cause of its occurrence. Autoimmune thrombocytolysis is called idiopathic when the cause of aggression against one’s own cells is not established, symptomatic if it is possible to establish why the blood platelets suddenly begin to collapse. Symptomatic thrombocytopenic purpura is often a companion to chronic pathological conditions:

• Scleroderma;

• Chronic forms (usually chronic lymphocytic leukemia);
• Inflammatory diseases of the liver and kidneys;

With AITP, the immune system suddenly begins not to recognize its own platelet, which is completely normal in all respects, and, mistaking it for a “stranger,” responds by producing antibodies against it.

Immune thrombocytopenic purpura occurs at any age, starting from the neonatal period, so it is far from uncommon in children. The disease primarily affects females. Flow pathological process often acquires a chronic relapsing form, especially for idiopathic thrombocytopenic purpura, since existing hypotheses of its occurrence do not explain the true cause of the disease.

Development of purpura

The development of thrombocytopenic purpura largely depends on thrombocytolysis (cell death under the influence of antibodies). In the bone marrow, megakaryocytes begin to be actively produced, which are quickly consumed and increase the number of platelets entering the bloodstream, where the blood platelets die within a short time. Instead of their allotted week of life, they exist for several hours, thereby forcing the bone marrow to work intensively and replenish losses. Such thrombocytopenias are called hyperregenerative, which mainly occur in children and make up the majority of clinical forms in pediatrics. But it happens that antibodies, in addition to platelets, are also directed at megakaryocytes, which devastates the sprout and prevents blood platelets from forming. This is the so-called hyporegenerative thrombocytopenia, which is not necessarily immune.

A large role in the pathogenesis of TP is given to the functional characteristics of platelets, their participation in hemostasis and nutrition of the vascular wall, as well as adhesive-aggregation ability, because they are able to stick to each other and to the damaged endothelium with the formation of a platelet plug.

It can be concluded that the main factor triggering bleeding is thrombocytopenia. When the vascular walls stop receiving platelet feeding, their degeneration occurs, which cannot prevent the passage of red blood cells through the vessels. The slightest injury in such cases can cause prolonged bleeding.

Diagnostics

A diagnosis such as thrombocytopenic purpura can be suspected if there are frequent and hemorrhagic petechial-spotted rashes, which differ from allergic ones in that they do not disappear with pressure. A low platelet count in a blood test confirms the diagnosis of TP.

When diagnosing TP in a hemostasiogram, you can get an increase in Duke bleeding time to 30 minutes or more and a decrease (less than 60%) in blood clot retraction, while Lee White coagulation will remain normal. Thrombocytopenic purpura is differentiated from hereditary thrombocytopathy (thrombocytopenia) using family history. Hereditary thrombocytopathy is characterized by a shortening of the life of blood platelets due to defective membranes or a lack of enzymes in the cells themselves.

Hemorrhagic rash means purpura

Thrombocytopenic purpura is characterized by the appearance of a petechial-spotted type of bleeding. And in case of major injuries, ecchymatoses can be observed. Thus, the symptoms of thrombocytopenic purpura can be presented as follows:

1. Hemorrhages that appeared at injection sites;
2. Severe bleeding from the mucous membranes (oral cavity, tonsils, pharynx);
3. Infection one to two weeks before the rash appears;
4. Body temperature is normal even in children and, with only rare exceptions, it can rise to low-grade fever;
5. Single or multiple spontaneous hemorrhages (sometimes after minor trauma);
6. Asymmetrical skin lesions, and of different sizes;
7. Hemorrhages vary in color: from purple (bright red) to blue-green and yellow;
8. Bruises resolve within 3 weeks;
9. Inconsistency of injury and hemorrhage;
10. The appearance of hemorrhages at night (during sleep);
11. The appearance of a hemorrhagic rash on the legs, arms and torso;
12. Bleeding from the nose and into natural cavities;
13. Gastrointestinal bleeding (black stool or red blood);
14. Bloody vomit that wears secondary character, as it occurs as a result of swallowing blood from the nose;
15. Anemia that develops due to constant blood loss;
16. Possible, which is a very dangerous symptom.

In addition, bleeding from the ears, hemoptysis and hemorrhage into the vitreous body of the eye, which led to complete blindness, were described in medical practice.

People often confuse thrombocytopenic purpura with Henoch-Schönlein purpura, which is characterized by a vasculitic purpuric type of rash, which is why the disease is called. What is confusing is that the rash is similar to the petechial macular rash seen in TP. Henoch-Schönlein disease can be described as follows:

• A bright red papular rash that fades over time and leaves bluish pigment spots;
• Itching sensation before the rash;
• Body temperature often rises;
• The rashes are located symmetrically on the legs and arms;
• Damage to the vessels of the kidneys (micro- and macrohematuria).

The rash in the case of hemorrhagic vasculitis is very similar to an allergic one, but it does not disappear with pressure. Henoch-Schönlein disease has a chronic course, where, in addition to the skin, joints can be affected, gastrointestinal tract, mucous membranes, therefore there are 4 forms of this disease:

1. Skin:
2. Abdominal;
3. Articular;
4. Mixed.

How to treat thrombocytopenic purpura?

If thrombocytopenic purpura is suspected, the patient should be hospitalized, because strict bed rest with similar disease required until platelets are restored to the minimum physiological level.

If there is bleeding, the first step is to use local (ε-aminocaproic acid, hemostatic sponge, thrombin, adroxon) and general (ascorutin and calcium chloride for intravenous administration) hemostatic agents. At the first stage therapeutic measures include corticosteroid therapy, which lasts up to 3 months.

There is not much interest in treating thrombocytopenic purpura by transfusion of platelet mass, due to the fact that the donor's platelets must still take root in the recipient, and not immunize him even more (individual selection is indicated), therefore, in case of severe anemia that has arisen against the background of blood loss, preference is given to washed red blood cells.

Splenectomy ( radical method) is performed at the second stage of treatment in cases of persistent bleeding, aseptic inflammation or threat of splenic rupture. However, if removal of the spleen also does not have an effect, then treatment is continued with small doses of corticosteroids. Although they will not restore the platelet count, they will at least reduce the risk of bleeding in the brain.

For such patients, barbiturates, caffeine, aspirin and other drugs that help reduce the platelet component in the blood are absolutely contraindicated, so the patient is strictly warned about this.

After completing the course of treatment and being discharged from the hospital, the patient is registered at the clinic at the place of residence for further observation. In this case, it is mandatory to sanitize all lesions chronic infection, and the oral cavity in particular. Deworming is also carried out.

Considering that thrombocytopenic purpura is not uncommon in children, part of the responsibility for the further course of the disease rests with the parents. A conversation is held with them about what can provoke a relapse of the disease (ARVI, exacerbation of focal infections). In addition, parents should know how to gradually introduce hardening, physical therapy and keep a food diary (elimination of allergenic foods). To protect the child from injury, he is exempt from school for this period and is advised to study at home.

A person is registered at a dispensary for recovery for at least 2 years. The prognosis of the disease, if it is not thrombotic thrombocytopenic purpura, is usually favorable.

Video: thrombocytopenia and hemorrhagic syndrome