[Microangiopathic anemia following thrombopenic purpura].
BACKGROUND
Chronic relapsing microangiopathic hemolytic anemia is rare in children. This report describes a case associated with thrombocytopenia following idiopathic thrombocytopenic purpura.


METHODS
A 4 year-old girl was admitted for acute idiopathic thrombocytopenic purpura (platelet count: 12,000/mm3) without anemia or fragmented red cells. The patient was given intravenous gammaglobulins without success, followed by prednisone (2 mg/kg/day). The platelet count was normalized, but decreased when the treatment was discontinued. The patient developed acute intracranial hypertension at the age of 5 yr 8 mo, following 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 admitted because of diffuse purpura, anemia and jaundice. Hematologic findings were: Hb 8.4 g/dl, reticulocytes 448,200/mm3, fragmented red cells 16%, platelets 15,000/mm3, WBC 22,400/mm3. Seroimmunologic investigation showed a high titer of antinuclear antibodies. Examination for viral etiology was negative. Intravenous gammaglobulins had a transient effect on platelets, reticulocytes and fragmented red cells. The patient was then given vincristine plus prednisone; they were only effective when high doses were used. A second intracerebral hemorrhage occurred when the patient was given low doses of drugs. After 3 other hematologic relapses, the vincristine was stopped without further complication.


CONCLUSIONS
The criteria for systemic lupus erythematous were not satisfied, despite the presence of antinuclear antibodies. A congenital deficiency of an unidentified plasma factor that reverses microangiopathic hemolysis and thrombocytopenia was not demonstrated in this patient, who could not be given fresh frozen plasma.
Perelman
S
S
Service de Pédiatrie Générale, Hôpital Robert-Debré, Paris.
Vilmer
E
E
Bachelot
C
C
Bourrillon
A
A
D000208:Acute Disease; D000743:Anemia, Hemolytic / Q000209:etiology* / Q000276:immunology; D000974:Antibodies, Antinuclear / Q000032:analysis; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D012008:Recurrence; D013921:Thrombocytopenia / Q000209:etiology
D000974:Antibodies, Antinuclear
Anémie microangiopathique succédant à un purpura thrombopénique idiopathique.