Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus

Vol.20 No.1

Case Report

Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus

Authors

Kuniyoshi Kamiya¹ , Kazuhiro Kurasawa¹ , Satoko Arai¹ , Reika Maezawa¹ , Ryosuke Hanaoka¹ , Kotaro Kumano¹ , Takeshi Fukuda¹

Pulmonary Medicine and Clinical Immunology, Dokkyo University, School of Medicine, 880 Kitakobayashi, Mibu Tochigi, 260-8670, Japan

Received:

14 April 2009

Accepted:

26 August 2009

Published online:

26 September 2009

Full Text

PDF　(member's only)

Abstract

We report the case of a patient with systemic lupus erythematosus (SLE) who first revealed hemophagocytic syndrome (HPS), which was treated successfully with glucocorticoid and intravenous cyclophosphamide. The patient then demonstrated refractory thrombotic thrombocytopenic purpura (TTP) with normal a disintegrin and metalloprotease with thrombospondin motifs (ADAMTS)-13 activity that responded well to rituximab. After rituximab treatment, the patient showed a flare of HPS that was controlled by additional intravenous cyclophosphamide treatment. This case showed that TTP with normal ADAMTS-13 activity is B-cell dependent and indicated that B-cell depletion might exacerbate some autoimmune conditions in SLE.

Key words

Systemic lupus erythematosus - Rituximab - Hemophagocytic syndrome - Thrombotic thrombocytopenic purpura - Regulatory B cell