Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus
| Autor: | Ryosuke Hanaoka, Kuniyoshi Kamiya, Satoko Arai, Kazuhiro Kurasawa, Takeshi Fukuda, Reika Maezawa, Kotaro Kumano |
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| Rok vydání: | 2009 |
| Předmět: |
medicine.medical_specialty
Cyclophosphamide Regulatory B cells Thrombotic thrombocytopenic purpura ADAMTS13 Protein Lymphohistiocytosis Hemophagocytic Antibodies Monoclonal Murine-Derived Rheumatology Refractory immune system diseases hemic and lymphatic diseases Internal medicine medicine Humans Lupus Erythematosus Systemic skin and connective tissue diseases B-Lymphocytes Lupus erythematosus Plasma Exchange Purpura Thrombotic Thrombocytopenic business.industry Antibodies Monoclonal Middle Aged medicine.disease Combined Modality Therapy ADAM Proteins Antirheumatic Agents Injections Intravenous Monoclonal Immunology Drug Therapy Combination Female Rituximab business Immunosuppressive Agents medicine.drug |
| Zdroj: | Modern Rheumatology. 20:81-85 |
| ISSN: | 1439-7609 1439-7595 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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