Thrombotic thrombocytopenic purpura in an asplenic patient with hereditary spherocytosis: Failure of plasmapheresis, antiplatelet therapy, and corticosteroids

David L. Becton*, Morris Kietzei, Watson C. Arnold, D. H. Berry

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder characterized by microangiopathic hemolysis, central nervous system and renal dysfunction, and a very poor prognosis. Recently, however, plasma exchange or infusion therapy has proven effective in the majority of patients with TTP. Wc report a patient who developed TTP several years after splenectomy for hereditary spherocytosis. Despite aggressive therapy with plasmapheresis (PP), plasma infusion, antiplatelet drugs, and corticosteroids, the patient had progression of TTP that eventually resulted in his death. The occurrence of TTP in an asplenic patient with an intrinsic red cell disorder, a previously unreported association, may predict a poor prognosis.

Original languageEnglish (US)
Pages (from-to)5-8
Number of pages4
JournalJournal of Pediatric Hematology/Oncology
Volume10
Issue number1
DOIs
StatePublished - Jan 1 1988

Keywords

  • Antiplatelet therapy
  • Plasmapheresis
  • Spherocytosis
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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