@article{b89d137ccc1543728f09760b6193f4eb,
title = "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.",
author = "Murali Chintagumpala and Richard Hurwitz and Moake, {Joel L.} and Mahoney, {Donald H.} and Steuber, {Charles P.}",
note = "Funding Information: Relatively few children have been reported to have some or all of the clinical and laboratory features of the single-episode, irregular, infrequent or the chronic, regular, relapsing type of thrombotic thrombocytopenic purpura. 18 One report described a 4-year-old boy who had a chronic, relapsing type of the disorder with recurrences at regular intervals; the presence of unusually large yon Willebrand factor multimers was noted in his plasma during periods of remission. 5 Another report summarized the clinical histories of two adult women with a chronic relapsing type of TTP that began in the neonatal period. 8 Both of these patients had unusually large vWF multimers in plasma samples taken during periods of remission in adulthood. 8 Supported in part by the Hematology/Oncology Fund, Texas Children's Hospital, and byNational Institutes of Health grant No. HL 35387 (J.L.M.). Submitted for publication April 29, 1991; accepted July 23, 1991. Reprint requests: M. M. Chintagumpala, MD, Texas Children's Hospital, 6621 Fannin (3-289), Houston, TX 77030. 9/20/32718 We studied two children in whom TTP or TTP-like syndromes developed during infancy. Because of the rarity of these disorders in infants and children, we describe these two patients with chronic relapsing disorder who have been successfully treated and maintained in prolonged remission by the prophylactic use of fresh-frozen plasma without concurrent plasmapheresis. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.",
year = "1992",
month = jan,
doi = "10.1016/S0022-3476(05)80596-7",
language = "English (US)",
volume = "120",
pages = "49--53",
journal = "Journal of Pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "1",
}