Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus
Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus
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DOI：
10.3109/s10165-009-0231-8
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发表时间：
2010-02
影响因子：
2.2
通讯作者：
K. Kamiya;K. Kurasawa;S. Arai;R. Maezawa;R. Hanaoka;K. Kumano;T. Fukuda
中科院分区：
文献类型：
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作者：
K. Kamiya;K. Kurasawa;S. Arai;R. Maezawa;R. Hanaoka;K. Kumano;T. Fukuda
我们报告一位系统性红斑狼疮患者，首次发现噬血细胞症候群，并以糖皮质激素及环磷酰胺静脉注射成功治疗。然后，该患者表现出难治性血栓性血小板减少性紫癜（TTP），具有正常的具有血小板反应蛋白基序的去整合素和金属蛋白酶（ADAMTS）-13活性，对利妥昔单抗反应良好。利妥昔单抗治疗后，患者出现HPS发作，通过额外的静脉环磷酰胺治疗得到控制。该病例表明，具有正常ADAMTS-13活性的TTP是B细胞依赖性的，并表明B细胞耗竭可能加重SLE中的某些自身免疫性疾病。
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.