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

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.
Authors	Kuniyoshi Kamiya, Kazuhiro Kurasawa, Satoko Arai, Reika Maezawa, Ryosuke Hanaoka, Kotaro Kumano, Takeshi Fukuda
Journal	Modern rheumatology (Mod Rheumatol) Vol. 20 Issue 1 Pg. 81-5 (Feb 2010) ISSN: 1439-7609 [Electronic] England
PMID	19784542 (Publication Type: Case Reports, Journal Article)
Chemical References	Antibodies, Monoclonal Antibodies, Monoclonal, Murine-Derived Antirheumatic Agents Immunosuppressive Agents Rituximab Cyclophosphamide ADAM Proteins ADAMTS13 Protein ADAMTS13 protein, human
Topics	ADAM Proteins (blood) ADAMTS13 Protein Antibodies, Monoclonal (adverse effects) Antibodies, Monoclonal, Murine-Derived Antirheumatic Agents (adverse effects) B-Lymphocytes (drug effects, physiology) Combined Modality Therapy Cyclophosphamide (administration & dosage, therapeutic use) Drug Therapy, Combination Female Humans Immunosuppressive Agents (therapeutic use) Injections, Intravenous Lupus Erythematosus, Systemic (complications, drug therapy) Lymphohistiocytosis, Hemophagocytic (blood, chemically induced, therapy) Middle Aged Plasma Exchange Purpura, Thrombotic Thrombocytopenic (blood, complications, drug therapy) Rituximab

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