Korean J Med.  2006 Aug;71(2):214-218.

Cyclophosphamide treatment in a patient with thrombotic thrombocytopenic purpura and lupus nephritis: report of one case

Affiliations
  • 1Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. ygkim@smc.samsung.co.kr
  • 2Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 3Department of Internal Medicine, Seoul Medical Center, Seoul, Korea.

Abstract

Thrombotic thrombocytopenic purpura is a rare but fatal complication of systemic lupus erythematosus. The diagnosis of thrombotic thrombocytopenic purpura as a syndrome distinct from systemic lupus erythematosus may be challenging particularly when thrombotic thrombocytopenic purpura is presented concomitantly with systemic lupus erythematosus. Early diagnosis and aggressive treatment including plasmapheresis would be required. However, recent reports have suggested that the use of cyclophosphamide may have a role. We describe a patient with systemic lupus erythematosus who was first presented with severe thrombotic thrombocytopenic purpura. Diagnosis was based on typical clinical features of thrombotic thrombocytopenic purpura and laboratory findings of active lupus nephritis. Renal biopsy also confirmed the coexistence of thrombotic thrombocytopenic purpura and diffuse proliferative lupus nephritis. Although prompt extensive plasmapheresis and high dose steroid therapy were performed, oliguric renal failure and thrombocytopenia persisted. After addition of cyclophosphamide to the treatment with plasmapheresis and steroid, clinical manifestations of thrombotic thrombocytopenic purpura and lupus nephritis were markedly improved.

Keyword

Lupus Erythematosus; Systemic; Purpura; Thrombotic thrombocytopenic; Plasmapheresis; Cyclophosphamide
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