慢性移植物抗宿主病相关性肌炎一例  

Chronic graft versus host disease related polymyositis:a case report and literature review

作　　者：薛胜利[1] 吴德沛[1] 孙爱宁[1] 唐晓文[1] 傅 赵晔[1] 马骁[1] 刘跃均[1] 王荧[1] 吴小津[1] 

机构地区：[1]苏州大学附属第一医院血液科江苏省血液研究所,215006

出　　处：《中华器官移植杂志》2006年第11期689-691,共3页Chinese Journal of Organ Transplantation

基　　金：江苏省医学重点人才基金资助项目(RC2002033)

摘　　要：目的总结1例异基因造血干细胞移植后并发与慢性移植物抗宿主病（cGVHD）相关的多发性肌炎的诊治体会。方法1例急性淋巴细胞白血病患者在处于完全缓解状态下接受同胞间供髓异基因造血干细胞移植，移植后采用环孢素A和甲氨蝶呤预防移植物抗宿主病（GVHD）。结果移植后11d，WBC〉0．5×10^9／L，移植后13d，血小板〉20×10^9／L；27d时，骨髓细胞染色体分析显示99％为供者型。移植后17d，发生Ⅰ度急性皮肤型GVHD，经静脉注射地塞米松及甲氨蝶呤后，GVHD被完全控制。移植后8个月，患者发生轻度肝脏cGVHD，经他克莫司及硫唑嘌呤治疗，效果不佳，血清肝酶升高，后改为他克莫司和西罗莫司治疗，血清肝酶逐渐下降，但肌酸激酶从9U／L上升至272U／L，随后患者出现全身乏力，并逐渐加重，上下肢近端处活动出现障碍，肌酸激酶升至3010U／L，股四头肌、肱二头肌的肌电图表现为肌源性损害，双侧大腿磁共振成像符合多发性肌炎表现，给予甲泼尼龙、血浆置换治疗，但无显著效果，患者突发阵发性呼吸困难，经抢救无效，患者死亡，死亡时肌酸激酶为21010U／L。结论多发性肌炎为cGVHD的一种较少见形式，累及重要肌组织者预后较差。Objective To summarize the diagnostic and therapeutic experience of a patient with chronic graft versus host disease （cGVHD） related polymyositis （PM） after allogeneic hematopoietic stem cell transplantation （allo-HSCT）. Methods A patient with acute lymphocytic leukemia in complete remission received sibling allo-HSCT, and cyclosporine and methotrexate were adopted to prevent GVHD. Results Eleven days after HSCT, WBC〉0. 5 ×10^9/L, 13 days after HSCT, PLT〉20 ×10^9/L; 27 days after HSCT, chromosome analysis of bone marrow cells showed 99 % donor type. Seventeen days after HSCT, Ⅰ° acute GVHD of skin occurred, and it was cured by intravenous injection of dexamethasone and methotrexate. Eight months after HSCT, cGVHD of liver happened. Although treated by tacrolimus and azathioprine, enzymes of liver were still elevated. At last, tacrolimus combined with sirolimus were used, and enzymes of liver subsided gradually. However, the serum creatine phosphokinase （CK） began to rise from 9 U/L to 3010 U/L, and fatigue all over the patient occurred. Finally, the symptom relapsed, and disability involved with the origin of limbs appeared. The electromyogram and magnetic resonance imaging of concerned muscles confirmed the PM diagno- sis. Although treated with methylprednisolone and plasma exchange, the patient died due to asphyxia, while the CK as high as 21 010 U/L. Conclusion PM is a rare kind of manifestations of cGVHD. When the key muscle tissue was involved, the prognosis is poor.

关 键 词：造血干细胞移植 移植物抗宿主病 多发性肌炎 

分 类 号：R733.71[医药卫生—肿瘤]