常规治疗无效的系统性红斑狼疮伴发难治性血小板减少的后续治疗策略（附3例报告）

Strategies for systemic lupus erythematosus patients with severe and refractory thrombocytopenia after failure to high-dose methylprednisolone, Cyclophosphamide pulse and intravenous immuno-globulin therapy：report of three cases

目的探讨系统性红斑狼疮（SLE）伴发严重难治性血小板减少的治疗策略。方法分析3例系统性红斑狼疮并严重难治性血小板减少患者的临床资料，并就治疗方案的选择进行讨论。结果3例患者均为女性，年龄范围28-38岁。1例严重血小板减少出现在SLE确诊5年后，1例为SLE的首发症状，另1例先有自身免疫性溶血性贫血，外科手术后出现明显的Evans综合征而诊断SLE。3例患者均对常规的大剂量甲泼尼龙和环磷酰胺冲击治疗以及静脉应用免疫球蛋白治疗无效，但对达那唑、小剂量环孢素和利妥昔单克隆抗体联合治疗或脾切除有所反应。结论对于常规治疗无效的SLE伴发严重难治性血小板减少的患者可采用小剂量多药联合治疗或脾切除。

Objective To explore the treatment strategy for systemic lupus erythematosus（SLE） patients with severe and refractory thrombocytopenia. Methods The clinical data of three SLE patients with severe and refractory thrombocytopenia was reported and the Strategies of alternative treatment was discussed. Results Three cases were female with a age range from 28 to 38 years old. one case presented severe thrombocytopenia as initial manefestation, one after 5 years of SLE diagnosis, and one as Evans syn- drome after surgery operation with a history of autoimmue hemolysis. All patients failed to high-dose meth- ylprednisolone, Cyclophosphamide plus and intravenous immunoglobulin（IVIG） treatment, but partial res- pose to low-dose Danazol, cyclosporine A （CsA） , and rituximab in combination or Splenectomy. Conclu- sion Low-dose Danazol, CsA, and rituximab in combination or Splenectomy can be used as alternative treatment for the SLE patients with severe and refractory thrombocytopenia.