Symptoms of thrombocytopenia in children
Thrombocytopenia in children is characterized by microcirculatory vascular type of bleeding: petechial hemorrhage, single or merging into extravasation, bleeding from mucous membranes and from injection sites, hemorrhages in the sclera, in the internal organs, including intracranial hemorrhages.
Isoimmune (alloimmune) trobocytopenia in children
Thrombocytopenia of the fetus and newborn due to the antigenic incompatibility of the mother and fetus platelets.
The disease is diagnosed in one newborn out of every 5,000 to 10,000. This pathology can occur both during the first and repeated pregnancies. Antigenic incompatibility occurs in the absence of maternal platelet antigens P1a1 (in 50% of cases of isoimmune thrombocytopenia) or Pb2, Pb3, Onro, Co., etc., which leads to isosensitization and the production of antithrombocytic antibodies to fetal platelets in the mother's body.
The clinical picture of isoimmune thrombocytopenia in children is characterized (immediately after the birth of the child) by petechial rash and small-blooded hemorrhages of the skin and mucous membranes. In severe cases (10-12% of patients), during the first hours and days of life, the hemorrhagic syndrome increases, melena, pulmonary, umbilical hemorrhage, and intracranial hemorrhages occur. Typical moderate splenomegaly. Characterized by severe thrombocytopenia, an increase in bleeding time. PV and APTTV are not changed, do not detect FDP. Thrombocytopenia persists for 4-12 weeks, gradually fading away.
The diagnosis is confirmed by the formulation of the thrombogaglutination reaction of the child's platelets in the mother's serum.
In 10-12% of cases, death is possible due to hemorrhage in vital organs, but in general the prognosis is favorable, the disease lasts for 3-4 months and gradually fades away until complete recovery.
Isoimmune thrombocytopenia therapy in children begins with the correct feeding of the newborn. For 2-3 weeks (depending on the severity of the disease) the child should be fed with donor milk or milk formulas.
Since the disease ends with spontaneous recovery after 3-4 months, drug treatment is indicated when the number of platelets is less than 20 × 10 9 / l and the presence of bleeding. Human immunoglobulin is prescribed as normal for intravenous administration at the rate of 800 mg / kg (daily drip, slowly, for 5 days) or at the rate of 1000-1500 mg / kg (1 time in 2 days, 2-3 times intravenously, drip, slowly).
Glucocorticoids are also used: prednisone 1-2 mg / (kg × days) by mouth (2/3 doses in the morning, 1/3 at 16 h) for 3-5 days.
In severe thrombocytopenia in children, transfusion of washed maternal platelets at a dose of 10–30 ml / kg or washed platelets of an antigen-negative donor (with an individual selection for antigen compatibility) is also effective at 10–30 ml / kg intravenously. To prevent the graft versus host reaction, blood components obtained from the patient’s relatives should be exposed to radiation.
In the absence of bleeding and moderate thrombocytopenia (platelet count not lower than 20-30 × 10 9 / l), sodium etamzilate (Dicynon) is administered intramuscularly or intravenously at a dose of 0.5-1.0 ml 1 time per day for 7-10 days. Also prescribed calcium pantothenate 0.01 g 3 times a day inside for 7-10 days.
Transimmune thrombocytopenia in children
Transimmune thrombocytopenia is transient thrombocytopenia in children born to mothers suffering from immune forms of thrombocytopenia (Verlhof disease and Fisher-Evans disease).
Transimmune thrombocytopenia occurs in 30-50% of children born to mothers suffering from these diseases (regardless of whether they have undergone splenectomy or not). The disease develops as a result of the transplacental transmission of maternal antiplatelet antibodies or a clone of sensitized lymphocytes, as a result of which thrombolysis and thrombocytopenia occur. More often (in 50% of cases) with transimmune thrombocytopenia in children they find an isolated decrease in the number of platelets, determined by laboratory and without clinical manifestations. When the platelet level is less than 50x10 9 / l, hemorrhagic syndrome of the microcirculatory type appears: petechial rashes, single extravasates. Bleeding from the mucous membranes and hemorrhages in the internal organs are rarely observed. The typical duration of hemorrhagic syndrome is 6-12 weeks.
Diagnosis is based on the presence of family history (thrombocytopenia in the mother). The platelet count in the blood is reduced, the bleeding time is increased, the clotting time, PV, APTT is normal. Anti-platelet antibodies are detected in the blood and breast milk of the mother (including in cases where the mother had previously had a splenectomy).
Treatment of transimmune thrombocytopenia in children begins with the correct feeding of the child (donor milk or milk formulas).
Drug therapy is indicated only for severe hemorrhagic syndrome. Human immunoglobulin used is normal for intravenous administration (800 mg / kg, 1-3 times), sodium etamsylate and prednisolone are also prescribed. In severe cases, the treatment regimen completely coincides with that in isoimmune thrombocytopenia of the newborn.
Heteroimmune thrombocytopenia in children
Heteroimmune thrombocytopenia in children is an immune form of thrombocytopenia, caused by the destruction of platelets under the influence of antibodies produced by the immune system of the child's body to platelets loaded with haptens of medicinal, microbial and viral origin.
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The causes of the disease are respiratory and other viruses, antibiotics (cefalotin, penicillin, ampicillin, rifampicin, chloramphenicol, erythromycin), thiazide diuretics (acetazolamide, furosemide), barbiturates. Adsorbed on the surface of red blood cells, these substances (haptens) stimulate the production of anti-erythrocyte antibodies, which leads to cell lysis.
Usually, after 2-3 days from the onset of a viral infection or taking a medicine, there appears scant microcirculatory hemorrhagic syndrome (petechiae, ecchymosis). Bleeding from the mucous membranes is rarely observed, there is no hemorrhage in the internal organs. The duration of hemorrhagic syndrome usually does not exceed 5-7 days.
Diagnosis of heteroimmune thrombocytopenia in children is based on anamnestic data: association with infection, prescription of drugs, development in the late neonatal period. The number of platelets is moderately reduced, the bleeding time is normal or slightly increased, the clotting time, PV, THT is normal.
Usually no treatment is required. The abolition of drugs is necessary, after which the hemorrhagic syndrome disappears within 2-5 days.
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Congenital hypo (a) megakaryocytosis
TAR syndrome (Thrombocytopenia-Absent Radii) - embryopathy in the form of atresia of the radius and thrombocytopenia in children due to hypo- or amegakaryocytosis.
The etiology and pathogenesis of the disease are not precisely established, for the development of the autosomal recessive form of the TAR syndrome, 11 genome microdeletions of chromosome lq21.1 are required, which leads to embryogenesis disorders at 7-9 weeks of gestation, resulting in hypo- or amegaryocytosis, atresia of the radius, malformations of the heart, kidneys and brain.
The clinical symptom complex includes atresia of both radial bones, various malformations and severe hemorrhagic syndrome of the microcirculatory type: multiple petechiae, ecchymosis, melena, hemorrhages from the kidneys and lungs, into the internal organs. The disease often leads to death in the neonatal period (from hemorrhage to vital organs) or in the first year of life (from various congenital malformations).
Laboratory characteristic of marked thrombocytopenia in children (up to single blood plates in the preparation), an increase in bleeding time at normal clotting time, normal PV and slightly elongated PTT, normal fibrinogen level, and the absence of FDP, which excludes DIC. On myelogram: hypomegakaryocytosis (up to single megakaryocytes in preparations). At the same time, there are no signs of leukemic infiltration and myelodysplastic syndrome.
In hypo (a) fibrinogenemia, a patient’s group blood thromboconcentrate is used (20–30 ml / kg intravenously, drip). If necessary, repeat the transfusion after 3-4 days. If the platelet count in the blood is less than 20,000 in 1 µl of blood, stem cells or bone marrow transplantation is performed.
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Congenital malformations - giant hemangioma in combination with thrombocytopenia and hemolytic anemia.
The reason for the formation of a giant hemangioma is not known, it causes deposition, sequestration and lysis of platelets and erythrocytes. In a laboratory study, a rapid decrease in platelet count and increased red blood cell lysis are found. A bleeding tendency, anemia and jaundice are clinically observed.
The diagnosis is established clinically. To assess the severity of the disease, platelet count, bilirubin level and degree of anemic syndrome are determined.
Surgical treatment. In preparation for the operation, correction of thrombocytopenia in children (transfusion of the thromboconcentrate) and anemia (transfusion of the erythrocyte mass) is necessary. Effective hormone therapy, prednisone prescribed in tablets of 4-8 mg / (kgshut), depending on body weight and age of the child. More often, the drug is taken every other day without reducing the dosage. Course duration 28 days. If necessary, after 6-8 weeks, repeat the course.
Anomaly of May-Hegglin
Hereditary autosomal dominant disease: moderate thrombocytopenia in children due to increased platelet lysis and, less commonly, microcirculatory hemorrhagic syndrome.
Clinically, an increased tendency to bleeding is observed during the pinching and inking procedures. In a laboratory study: large platelet sizes - up to 8-12 microns (giant platelets), moderate thrombocytopenia, changes in the morphology of platelets and neutrophils. The abnormal size of the platelets is the reason for their increased lysis. At the same time, basophil inclusions are determined in neutrophils (Gyöle body). No treatment is required.
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Thrombocytopenia in children with congenital and acquired neonatal infections
Thrombocytopenia in children with congenital and acquired neonatal infections manifest hemorrhagic syndrome not associated with DIC, which often occurs in severe infectious diseases (both viral and bacterial) of the newborn.
Thrombocytopenia in the neonatal period is found in 10-15% of cases of severe infections. Their most common cause is congenital cytomegalovirus infection. Less commonly, thrombocytopenia occurs in congenital toxoplasmosis, syphilis, as well as herpes virus and enterovirus infections. From acquired diseases, sepsis, necrotizing enterocolitis, phlegmon, and peritonitis can cause thrombocytopenia. Causes of thrombocytopenia in severe infections without the development of DIC syndrome: hypersplenism, leading to sequestration and lysis of platelets, suppression of platelet exfoliation from megakaryocytes, increased destruction of platelets due to fixation of toxins on them and increased consumption of platelets when endothelial damage is damaged. Each of these factors or their combinations cause a decrease in the number of platelets, which leads to the development of hemorrhagic syndrome.
The clinical picture of the disease depends on the underlying pathology and is complicated by the hemorrhagic syndrome of the microcirculatory-vascular type (petechiae, hematomas at injection sites, bleeding from the mucous membranes, more often the gastrointestinal tract). Hemorrhagic syndrome is transient, easily reversible.
In a laboratory study, a decrease in the number of platelets is found, an increase in bleeding time during normal clotting time, TV and PTT, the amount of PDF in the blood is not elevated, which distinguishes thrombocytopenia in children with infections from DIC.
Special treatment is usually not required. Adequate therapy of the underlying disease is required. In severe bleeding and platelet levels of less than 20 × 10 9 / l, replacement transfusion of the patient’s blood group thrombocontinent (10–30 ml / kg, intravenous drip) is indicated.