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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11 d,WBC＞0．5×10~9/L,移植后13 d,血小板＞20×10~9/L；27 d时,骨髓细胞染色体分析显示99%为供者型。移植后17 d,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9 U/L上升至272 U/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3010 U/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21 010 U/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 diagnosis and treatment of polymyositis associated with chronic graft-versus-host disease (cGVHD) after one allogeneic hematopoietic stem cell transplantation. Methods One patient with acute lymphoblastic leukemia received sibling allogeneic hematopoietic stem cell transplantation in complete remission. After transplantation, cyclosporin A and methotrexate were used to prevent graft versus host disease (GVHD). Results On the 11th day after transplantation, WBC> 0.5 × 10 ~ 9 / L, 13 days after transplantation, and platelet count> 20 × 10 ~ 9 / L. On day 27, 99% of the bone marrow cells showed donor type. At 17 days after transplantation, grade 1 acute skin-type GVHD occurred. After dexamethasone and methotrexate were administered intravenously, GVHD was completely controlled. At 8 months after transplantation, mild hepatic cGVHD occurred in the patient. After tacrolimus and azathioprine treatment, the effect was poor and the serum liver enzyme was elevated. After treatment with tacrolimus and sirolimus, the serum liver Enzyme gradually decreased, but creatine kinase increased from 9 U / L to 272 U / L, then patients with generalized weakness and gradually increased, upper and lower extremity activities appear obstacles, creatine kinase increased to 3010 U / L, Unit Quadriceps, biceps EMG showed myogenic damage, bilateral thigh MRI consistent with polymyositis performance, given methylprednisolone, plasma exchange treatment, but no significant effect in patients with burst array Paroxysmal dyspnea, the rescue was invalid, the patient died, creatine kinase at the death of 21 010 U / L. Conclusions Polymyositis is a less common form of cGVHD, with poor prognosis involving important muscle tissues.