Chronic relapsing thrombotic thrombocytopenic purpura in infants with large von Willebrand factor multimers during remission.

Abstract

We studied two children with recurrent schistocytic hemolytic anemia and thrombocytopenia beginning in the neonatal period. One patient had a stroke during one of the episodes of thrombotic thrombocytopenic purpura. The presence of unusually large von Willebrand factor multimers was demonstrated in both children during clinical and hematologic remissions. Treatment with corticosteroids and intravenous injections of immune globulin was unsuccessful in the one patient who received it. Immediate improvement occurred in both patients after the infusion of fresh-frozen plasma. Symptoms of thrombocytopenia continue to recur at regular intervals in the absence of periodic fresh-frozen plasma infusions. One of these children apparently has chronic relapsing thrombotic thrombocytopenic purpura; the second has a chronic relapsing disorder similar to thrombotic thrombocytopenic purpura.

• Authors: M M Chintagumpala, R L Hurwitz, J L Moake, D H Mahoney, C P Steuber
• Journal: The Journal of pediatrics (J Pediatr) Vol. 120 Issue 1 Pg. 49-53 (Jan 1992) ISSN: 0022-3476 [Print] United States
• PMID: 1731024 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Hemoglobins
• von Willebrand Factor

Topics

• Blood Component Transfusion
• Chronic Disease
• Exchange Transfusion, Whole Blood
• Hemoglobins (analysis)
• Humans
• Hyperbilirubinemia (therapy)
• Infant
• Infant, Newborn
• Male
• Plasma
• Platelet Count
• Purpura, Thrombotic Thrombocytopenic (blood, drug therapy)
• Recurrence
• Remission Induction
• von Willebrand Factor (analysis)