血浆置换联合化疗、免疫抑制剂治疗噬血细胞综合症合并血栓性血小板减少性紫癜的临床观察

Clinical observation of the combination of plasmapheresis with chemotherapy and immunosuppressants for treatment of patients with HPS and TTP

目的观察血浆置换联合化疗、免疫抑制剂治疗罕见病——噬血细胞综合征合并血栓性血小板减少性紫癜的疗效。方法 2014年1-3月对本科两例噬血细胞综合征合并血栓性血小板减少性紫癜患者采用血浆置换联合环孢素、糖皮质激素、足叶乙甙方案进行治疗,随访至2015年3月。结果 2例患者血常规均恢复正常,发热、精神症状消失,肾功能恢复正常,骨髓嗜血现象消失,至今存活。结论血浆置换联合化疗、免疫抑制剂是治疗噬血细胞综合征合并血栓性血小板减少性紫癜的有效方法。

Objective To investigate the effect of the combination of plasmapheresis with chemotherapy and immunosuppressants for treatment of patients with hemophagocytic syndrome（ HPS） and thrombotic thrombocytopenic purpura（ TTP）. Methods Two patients with HPS and TTP were salvaged by plasmapheresis combined with chemotherapy and immunosuppressants during January and March of 2014. Results After treatment,patients＇ temperature,blood cell count and renal function returned to normal; mental symptoms and hemophagocytic bone marrow phenomenon disappeared. Two patients have survived until now. Conclusion It is suggested that plasmapheresis combined with chemotherapy and immunosuppressants is an effective regimen for treatment of patient with HPS and TTP.