Werlhof's disease (Verlgof's Disease)

Description:

Werlhof's disease (Verlgof's disease) - the disease which is characterized by tendency to bleeding caused by thrombocytopenia (decrease in maintenance of thrombocytes in blood to 150×109/л) at the normal or increased quantity of megacaryocytes in red marrow.
Werlhof's disease - the most widespread disease from group of hemorrhagic diathesis. Frequency of identification of new cases of a Werlhof's disease makes from 10 to 125 on 1 million population a year. The disease, as a rule, demonstrates at children's age. To 10-year age the disease meets identical frequency at boys and girls, and after 10 years and at adults - is 2-3 times more often at persons of a female.

Symptoms of the Werlhof's disease (Verlgof's Diseases):

The disease begins gradually or sharply with emergence of a hemorrhagic syndrome. Bleeding type at a Werlhof's disease petekhialno-spotty (sinyachkovy). On clinical manifestations allocate two options of a Werlhof's disease: Sukhoi - at the patient arises only a skin hemorrhagic syndrome; "wet" - hemorrhages in combination with bleedings. Pathognomonic symptoms of a Werlhof's disease - hemorrhage in skin, mucous membranes and bleedings. Lack of these signs raises doubts in correctness of the diagnosis.

    * The skin hemorrhagic syndrome arises at 100% of patients. The quantity of ecchymomas varies from single to multiple. The main characteristics of a skin hemorrhagic syndrome at a Werlhof's disease the following.
          - Discrepancy of expressiveness of a hemorrhage of extent of traumatic influence; their spontaneous emergence is possible (preferential at night).
          - Polymorphism of hemorrhagic rashes (from petechias to large hemorrhages).
          - The Polikhromnost skin hemorrhages (coloring from crimson to blue-greenish and yellow depending on prescription of their emergence) that is connected with gradual transformation of hemoglobin through intermediate stages of disintegration in bilirubin.
          - Asymmetry (there is no favourite localization) hemorrhagic elements.
          - Painlessness.
    * Quite often there are hemorrhages in mucous membranes, most often almonds, a soft and hard palate. Hemorrhages in a tympanic membrane, a sclera, a vitreous, an eyeground are possible.
    * Hemorrhage in a sclera can indicate threat of emergence of the heaviest and dangerous complication of a Werlhof's disease - hemorrhage in a brain. As a rule, it arises suddenly and quickly progresses. Clinically hemorrhage in a brain is shown by a headache, dizziness, spasms, vomiting, focal neurologic symptomatology. The outcome of a hematencephalon depends on volume, localization of pathological process, timeliness of diagnosis and adequate therapy.
    * Bleedings from mucous membranes are characteristic of a Werlhof's disease. Quite often they have profuse character, causing heavy posthemorrhagic anemia, life-threatening the patient. Children most often have bleedings from a mucous membrane of a nasal cavity. Odontorrhagias are usually less plentiful, but also they can become dangerous at extraction of teeth, especially at patients with not diagnosed disease. Bleeding after an odontectomy at a Werlhof's disease arises at once after intervention and does not renew after its termination unlike the late, delayed bleedings at hemophilia. At girls of the pubertal period also metrorrhagias are possible heavy meno-. More rare there are gastrointestinal and renal bleedings.
Characteristic changes of internals at a Werlhof's disease are absent. Body temperature is usually normal. Sometimes reveal tachycardia, at heart auscultation - systolic noise on a top and in Botkin's point, easing of the I tone, caused by anemia. Increase in a spleen uncharacteristically and excludes the diagnosis of a Werlhof's disease rather.
On a current allocate acute (lasting up to 6 months) and chronic (duration more than 6 months) disease forms. At primary survey it is impossible to establish character of a course of a disease. Depending on extent of manifestation of a hemorrhagic syndrome, blood indicators during a disease allocate three periods: hemorrhagic crisis, clinical remission and kliniko-hematologic remission.

    * Hemorrhagic crisis is characterized by the expressed bleeding syndrome, considerable changes of laboratory indicators.
    * During clinical remission the hemorrhagic syndrome disappears, the bleeding time is reduced, secondary changes in coagulant system of blood decrease, but thrombocytopenia remains, though it less expressed, than at hemorrhagic crisis.
    * Kliniko-gematologichesky remission means not only lack of bleeding, but also normalization of laboratory indicators.

Reasons of the Werlhof's disease (Verlgof's Diseases):

At a Werlhof's disease thrombocytopenia develops owing to destruction of thrombocytes by means of immune mechanisms. Antibodies to own thrombocytes can appear later 1-3 weeks after the postponed viral or bacterial infections, preventive inoculations, reception of medicines at their individual intolerance, overcoolings or insolations, after surgeries, injuries. In some cases any certain reason does not manage to be revealed. The antigens which came to an organism (for example, viruses, medicines, including vaccines) accumulate on thrombocytes of the patient and induce an immune response. Antithrombocytic antibodies carry preferential to IgG. Reaction of "Ag-AT" happens on a surface of thrombocytes. Life expectancy of the thrombocytes loaded with antibodies at a Werlhof's disease is reduced till several o'clock instead of 7-10 days normal. Premature death of thrombocytes happens in a spleen. Bleeding at a Werlhof's disease is caused by decrease in quantity of thrombocytes, secondary damage of a vascular wall in connection with loss of angiotrofichesky function of thrombocytes, disturbance of sokratitelny ability of vessels because of decrease in concentration of serotonin in blood, impossibility of retraction of a blood clot.

Treatment of the Werlhof's disease (Verlgof's Diseases):

In the period of hemorrhagic crisis the bed rest with its gradual expansion in process of fading of the hemorrhagic phenomena is shown to the child. The special diet is not appointed, however at bleeding of a mucous membrane of an oral cavity children have to receive chilled food.
Pathogenetic therapy of an autoimmune Werlhof's disease includes purpose of glucocorticoids, performance of a splenectomy and use of immunodepressants.

    * Prednisolonum appoint in a dose 2 mg/kg/days to 2-3 weeks with the subsequent a dose decline and full drug withdrawal. Prednisolonum in higher doses (3 mg/kg/days) is appointed short courses for 7 days with a break in 5 days (no more than three courses). At sharply expressed hemorrhagic syndrome, threat of a hematencephalon it is possible "pulse therapy" Methylprednisolonum (30 mg/kg/days intravenously within 3 days). In most cases this therapy is quite effective. In the beginning the hemorrhagic syndrome disappears, then the maintenance of thrombocytes begins to increase. At some patients after cancellation of hormones there comes the recurrence.
    * In recent years in treatment of a Werlhof's disease with good effect use intravenous administration of Ig human normal in a dose of 0,4 or 1 g/kg within 5 or 2 days respectively (a course dose of 2 g/kg) as monotherapy or in combination with glucocorticoids.
    * A splenectomy or thromboembolization of vessels of a spleen will see off at absence or instability of effect of conservative treatment, the repeating plentiful long bleedings leading to the expressed posthemorrhagic anemia, heavy bleedings life-threatening the patient. Operation is usually carried out against the background of glucocorticoid therapy at children 5 years as at earlier age the risk of development of postpneumonectomy sepsis is high are more senior. At 70-80% of patients operation leads to almost absolute recovery. Other children and after a splenectomy need treatment continuation.
    * Immunodepressants (cytostatics) for treatment of a Werlhof's disease at children use only in the absence of effect of other types of therapy as efficiency of their use is much less, than a splenectomy. Apply Vincristinum in a dose of 1,5-2 mg/sq.m of a body surface inside, cyclophosphamide in a dose of 10 mg/kg - 5-10 injections, Azathioprinum in a dose of 2-3 mg/kg/days in 2-3 receptions within 1-2 months.
Recently apply to treatment of a Werlhof's disease also даназол (synthetic drug of androgenic action), interferon drugs (реаферон, intron-A, roferon-A), anti-D - Ig (anti-D). However positive effect from their use unstable, side effects are possible that does necessary further studying of the mechanism of their action and definition of their place in complex therapy of this disease.
For reduction of expressiveness of a hemorrhagic syndrome in the period of the raised bleeding appoint intravenously or inside aminocapronic acid at the rate of 0,1 g/kg (it is contraindicated at a hamaturia). Drug belongs to fibrinolysis inhibitors, and also strengthens aggregation of thrombocytes. Apply also a hemostatic etamsylate in a dose of 5 mg/kg/days inside or intravenously kapelno. Drug possesses also angioprotektorny and proagregantny action. For a stop of nasal bleedings use tampons with hydrogen peroxide, adrenaline, aminocapronic acid; absorbable gelatin sponge, fibrinny, gelatinous films.
At treatment of posthemorrhagic anemia at children with a Werlhof's disease apply the means stimulating a hemopoiesis as regenerator abilities of the hemopoietic system at this disease are not broken. Transfusion of the washed erythrocytes which are picked up individually is carried out only at the expressed acute anemia.