【摘要】 目的分析肝移植术后患者发生噬血细胞综合征(hemophagocytic syndrome,HPS)的危险因素及其临床特点、诊断和预后。方法回顾性分析中山大学附属第三医院肝移植中心772例肝移植受者中发生的1例HPS患者的临床资料,并结合已经报道的13例肝移植术后HPS的临床资料进行文献复习。结果患者,男性、45岁,在肝移植术后36d发生持续性高热、全血细胞减少、凝血功能障碍和血清铁蛋白的水平升高。根据临床表现、实验室检查和骨髓活组织检查结果诊断为HPS。给予积极的肾上腺皮质激素冲击、注射粒细胞集落刺激因子和大剂量的免疫球蛋白治疗,因病情凶险,患者于术后54d死于颅内出血和多器官功能衰竭。在14例肝移植术后HPS患者中,临床表现为发热(14/14)、脾肿大(8/14);实验室检查发现贫血、血小板减少、白细胞减少、肝脏转氨酶升高(9/14);71%的患者血清铁蛋白水平升高。HPS的发生与病毒感染(8/14)有关。尽管给予积极的抗感染治疗和免疫抑制剂的减量,但仍有9例患者在术后2个月内死亡。结论肝移植术后HPS的发生与病毒尤其是爱泼斯坦-巴尔病毒(Epstein-Barruirus,EBV)和巨细胞病毒(CMV)感染有关。临床上疑似HPS时,应反复进行骨髓涂片检查,有助于早期确诊。尽管早期积极的合理治疗能够一定程度上控制病情,但总体预后极差。更多还原

【Abstract】 Objective To analyze the risk factors, clinical characteristics, diagnosis and prognosis of hemophagocytic syndrome (HPS) after liver transplantation. Methods In 772 patients underwent liver transplantation in Liver Transplantation Center of the Third Affiliated Hospital of Sun Yat-sen University, clinical data of one case who developed HPS were retrospectively analyzed.And clinical data of other 13 cases which had already reported and related literatures were reviewed. Results The patient, a 45-year-old man who developed HPS at day 36 after liver transplantation, had high fever, pancytopenia, coagulation disorders and hyperferritinemia.HPS was diagnosed according to clinical manifestations, laboratory data and bone marrow biopsy. Management with high-dose steroid pulse, administration of granulocyte colony-stimulating factor(G-CSF)and intravenous immunoglobulin supplementation failed to control his critical condition, and the patient died of intracranial hemorrhage and multiple organ failure on day 54 after liver transplantation.In 14 patients with HPS after liver transplantation, the clinical manifestations included fever(14/14)and splenomegaly(8/14). Laboratory tests showed anemia, thrombocytopenia, leucopenia and increase of hepatic aminotransferase(9/14). Serum ferritin level increased in 71% of patients.The occurrence of HPS was related to viral infection(8/14). Despite active anti-infectious treatment and dramatic tapering of immunosuppression, 9 patients died within 2 months after transplantation. Conclusions HPS after liver transplantation is usually associated with viral infections, especially Epstein-Barr virus and cytomegalovirus.When HPS is suspected, bone marrow smear should be performed and repeated if necessary.Although active treatment in early stage is necessary to control the condition, the total prognosis remains poor.更多还原