治愈肝移植术后噬血细胞综合征1例报告

A Successful Case Report of Hemophagocytic Syndrome After Liver Transplantation

目的:分析肝移植术后噬血细胞综合征(HPS)发生的危险因素及其临床表现、诊断和预后。方法:回顾性分析武汉大学移植医学中心208例肝移植受者中发生的1例HPS的患者的临床资料,并结合目前HPS的研究进展进行文献复习。结果:51岁男性患者,因胆汁淤积性肝硬化行同种异体肝移植术,术后并发肺部感染,持续高热,全血细胞减少,血甘油三酯增高,血清铁蛋白增高,骨髓涂片呈现噬血征象,诊断为噬血细胞综合征。积极抗感染治疗、甲强龙冲击治疗、大剂量免疫球蛋白冲击治疗及营养支持治疗后,患者感染得到控制,一般情况恢复良好后出院,现术后2年,健康存活,无后遗症。结论:肝移植术后HPS的发生与感染及免疫状态密切相关,极其凶险,患者总体预后差,临床上疑似HPS时,应进行骨髓涂片检查以早期诊断,早期确诊有助于及时调整合适的治疗方案,提高患者的生存率。

Objective： To analyze the risk factors of hemophagocytic syndrome （HPS） after liver trans- plantation and the clinical features, diagnosis and prognosis. Methods. We retrospectively ana- lyzed the clinical data of one patient with HPS in 208 liver transplant recipients from the Trans- plant Center of Wuhan University, and reviewed the study progress of HPS. Results： A 51-year- old male patient, underwent allograft liver transplantation due to cholestatic cirrhosis, postopera- tive pulmonary infection appeared, sustained high fever, reduced blood ceils, increased blood tri glycerides and increased serum ferritin were observed, bone marrow smear showed hemophago cytic sign, then diagnosis of HPS was made. The infection was controlled and patient survived af- ter active anti-infective treatments, methylprednisolone stosstherapy, high-dose immunoglobulintreatment and nutritional support treatment, after 2 years after surgery, the patient is living healthily, with no sequelae. Conclusion： HPS after liver transplantation is extremely dangerous and closely related to infection and immune status, always with poor prognosis, bone marrow smear should be carried out when HPS is suspected, early diagnosis helps a lot to adjust the ap- propriate treatment, and improve the survival rate of patients.