title: Lupus-associated thrombotic thrombocytopenic purpura-like microangiopathy.
abstract: Recently reported cases of lupus complicated by a thrombotic thrombocytopenic purpura (TTP)-like syndrome suggest a survival benefit to early treatment with plasma exchange. The following is a report of the eighth such case in the last ten years. A 44-year-old lady known for lupus presented with the nephrotic syndrome and a renal biopsy was consistent with class 4G lupus nephritis. She was given high-dose steroids and cytotoxic therapy, but her induction therapy was complicated by the classic pentad of TTP. She was subsequently treated with another course of high-dose steroids, a different cytotoxic agent, and plasma exchange, with clinical resolution shortly thereafter. Similar to seven recently reported cases of microangiopathy in lupus, this lady's TTP-like syndrome improved dramatically after initiation of plasma exchange, despite not having a severely deficient ADAMTS13. This has implications on both current clinical practice and on the pathogenesis of TTP-like syndromes in lupus.
authors.affiliation: Daniel Blum, Geoffrey Blake, Department of Hematology, McGill University Health Center, Montreal, Quebec H3G1A4, Canada.
authors.forename: Daniel
authors.initials: D
authors.lastname: Blum
authors.affiliation: Daniel Blum, Geoffrey Blake, Department of Hematology, McGill University Health Center, Montreal, Quebec H3G1A4, Canada.
authors.forename: Geoffrey
authors.initials: G
authors.lastname: Blake
keywords: Atypical hemolytic-uremic syndrome; Hemolytic uremic syndrome; Lupus; Lupus nephritis; Microangiopathic hemolytic anemia; Microangiopathy; Plasma exchange; Systemic lupus erythematosus associated thrombotic thrombocytopenic purpura-like microangiopathic hemolytic anemia; Thrombotic thrombocytopenic purpura