Thrombotic Thrombocytopenic Purpura Syndrome in Systemic Lupus Erythematosus: Treatment With Plasma Infusion

Janice Gelfand, Luan Truong, Leonard Stern, Conrad L. Pirani, Gerald B. Appel

Research output: Contribution to journalArticle

43 Scopus citations

Abstract

Two patients with well documented systemic lupus erythematosus developed a syndrome resembling thrombotic thrombocytopenic purpura. Both had severe thrombocytopenia, microangiopathic hemolytic anemia, seizures, and renal dysfunction. Prothrombin time, partial thromboplastin time, thrombin time, and fibrinogen levels were normal; fibrin degradation products were minimally elevated. Histologic evaluation of renal biopsies in both patients confirmed the impression of intravascular thrombosis. Therapy with corticosteroids, other immunosuppressive drugs and splenectomy (in one case) proved unsuccessful. The infusion of fresh frozen plasma, with or without plasmapheresis, reversed the syndrome. This report indicates that patients with systemic lupus may develop a thrombotic thrombocytopenic purpura like syndrome which responds to fresh plasma infusion.

Original languageEnglish (US)
Pages (from-to)154-160
Number of pages7
JournalAmerican Journal of Kidney Diseases
Volume6
Issue number3
DOIs
StatePublished - Jan 1 1985

Keywords

  • Thrombotic thrombocytopenic purpura
  • plasma infusion
  • systemic lupus erythematosus

ASJC Scopus subject areas

  • Nephrology

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