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Modern Rheumatology
Published in: Modern Rheumatology 1/2010

01-02-2010 | 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 Kamiya, Kazuhiro Kurasawa, Satoko Arai, Reika Maezawa, Ryosuke Hanaoka, Kotaro Kumano, Takeshi Fukuda

Published in: Modern Rheumatology | Issue 1/2010

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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.
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Metadata
Title
Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus
Authors
Kuniyoshi Kamiya
Kazuhiro Kurasawa
Satoko Arai
Reika Maezawa
Ryosuke Hanaoka
Kotaro Kumano
Takeshi Fukuda
Publication date
01-02-2010
Publisher
Springer Japan
Published in
Modern Rheumatology / Issue 1/2010
Print ISSN: 1439-7595
Electronic ISSN: 1439-7609
DOI
https://doi.org/10.1007/s10165-009-0231-8

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