Pulmonary Medicine and Clinical Immunology, Dokkyo University, School of Medicine, 880 Kitakobayashi, Mibu, Tochigi, 260-8670, Japan.
Mod Rheumatol. 2010 Feb;20(1):81-5. doi: 10.1007/s10165-009-0231-8. Epub 2009 Sep 26.
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.
我们报告了一例系统性红斑狼疮（SLE）患者，其最初表现为噬血细胞综合征（HPS），经糖皮质激素和静脉注射环磷酰胺治疗后成功缓解。随后，该患者出现难治性血栓性血小板减少性紫癜（TTP），但 ADAMTS-13 活性正常，对利妥昔单抗反应良好。利妥昔单抗治疗后，患者出现 HPS 加重，经额外静脉注射环磷酰胺治疗后得到控制。该病例表明，ADAMTS-13 活性正常的 TTP 依赖于 B 细胞，并且表明 B 细胞耗竭可能会加重 SLE 中的某些自身免疫状况。
