MICROANGIOPATHIC HEMOLYTIC-ANEMIA AFTER I DIOPATHIC THROMBOCYTOPENIC PURPURA

Background. Chronic relapsing microangiopathic hemolytic anemia is rar e in children. This report describes a case associated with thrombocyt openia following idiopathic thrombocytopenic purpura. Case report. A 4 year-old girl was admitted for acute idiopathic thrombocytopenic purp ura (platelet count : 12,000/mm3) without anemia or fragmented red cel ls. The patient was given intravenous gammaglobulins without success, followed by prednisone (2 mg/kg/day). The platelet count was normalize d, but decreased when the treatment was discontinued. The patient deve loped acute intracranial hypertension at the age of 5 yr 8 mo, followi ng two cerebral hematomas. The platelet count was 9,000/mm3. A second course of intravenous gammaglobulins and prednisone was unsuccessful, so a splenectomy was performed. One year later, the patient was admitt ed because of diffuse purpura, anemia and jaundice. Hematologic findin gs were : Hb 8.4 g/dl, reticulocytes 448,200/mm3, fragmented red cells 16 %, platelets 15,000/mm3, WBC 22,400 mm3. Seroimmunologic investiga tion showed a high titer of antinuclear antibodies. Examination for vi ral etiology was negative. Intravenous gammaglobulins had a transient effect on platelets, reticulocytes and fragmented red cells. The patie nt was then given vincristine plus prednisone ; they were only effecti ve when high doses were used. A second intracerebral hemorrhage occurr ed when the patient was given low doses of drugs. After 3 other hemato logic relapses, the vincristine was stopped without further complicati on. Conclusion. The criteria for systemic lupus erythematous were not satisfied, despite the presence of antinuclear antibodies. A congenita l deficiency of an unidentified plasma factor that reverses microangio pathic hemolysis and thrombocytopenia was not demonstrated in this pat ient, who could not be given fresh frozen plasma.