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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 and their clinical manifestations, diagnosis and prognosis of hemophagocytic syndrome (HPS) after liver transplantation. Methods: The clinical data of 208 patients with HPS who had undergone liver transplantation in the Transplantation Center of Wuhan University were analyzed retrospectively. The literature review was made based on the current research progress of HPS. Results: The 51-year-old male patient underwent allogeneic liver transplantation due to cholestatic cirrhosis. Postoperative pulmonary infection, persistent fever, pancytopenia, elevated triglyceride, increased serum ferritin, bone marrow smear Hemophagocytic signs, the diagnosis of hemophagocytic syndrome. Active infection control, methylprednisolone therapy, high-dose immunoglobulin shock therapy and nutritional support treatment, the patient’s infection was controlled, the general situation was recovered well after discharge, now 2 years after surgery, healthy survivors, no sequelae. Conclusion: The incidence of HPS after liver transplantation is closely related to the infection and immune status. It is extremely dangerous. The overall prognosis of patients with HPS is poor. In the case of clinically suspected HPS, bone marrow smears should be used for early diagnosis. Early diagnosis can help to timely adjust appropriate Treatment options to improve patient survival.