目的 探讨对成人不明原因嗜血综合征病人开展脾脏切除术的安全性与可行性。方法 回顾分析2013年1月至2017年1月在复旦大学附属华东医院胆胰疾病诊疗中心接受脾脏切除术的29例成人不明原因嗜血综合征病人的手术及围术期临床资料,通过脾脏病理检查明确嗜血综合征病因。结果 所有病人术前均有反复高热,27例（93.1%）的病人二系或三系血细胞异常,23例（79.3%）肝功能异常,24例（82.8%）凝血功能异常。所有病人术前美国麻醉师协会评分均在Ⅲ级以上。脾脏最大径（22.8±6.3） cm,手术时间（112.3±27.7） min,出血量（281.8±399.5） mL。术后胰漏8例（27.6%）,其中A级胰漏5例(17.2%),B级3例（10.3%）。术后肺部感染3例（10.3%）,术后腹腔出血1例（3.5%）再次手术。无围术期死亡病例。27例（93.1%）病人明确诊断为淋巴瘤,2例（6.9%）未能明确嗜血综合征病因。结论 虽然成人嗜血综合征病人病情危重,存在多器官系统功能不全,但施行脾脏切除术是安全可行的。通过切除脾脏的病理检查能确诊嗜血综合征病因,指导随后的治疗方案。
Objective To evaluate the safety and feasibility of splenectomy in adult hemophagocytic lymphohistiocytosis (HLH) of unknown origin. Methods The clinical data of 29 adults with HLH of unknown origin treated with splenectomy in our center from January 2013 to January 2017 were reviewed retrospectively. The patients with HLH were diagnosed with pathological examination of spleen postoperatively. Results All cases had repeated high fever, 27 cases (93.1%) with cytopenia involving at least two or three lineages, 23 cases(79.3%) with abnormal liver function and 24 cases (82.8%) with dysfunction of blood coagulation. American Society of Anesthesiologists (ASA) scores were above grade Ⅲ in all patients before operation. The maximum diameter of spleen was (22.8± 6.3) cm. The operative time was(112.3± 27.7) min and the estimated blood loss (281.8± 399.5) mL. The postoperative pancreatic leak occurred in 8 cases (27.6%) according to ISGPF definition, including 5 cases (17.2%) of grade A and 3 cases(10.3%) of grade B. Three cases (10.3%) had pulmonary infection. One case (3.5%) required relaparotomy due to postoperative abdominal bleeding. There was no peri-operative mortality in this study. After splenectomy, a clear lymphoma diagnosis could be determined in 27 patients (93.1%) and the cause of HLH in other 2 patients was still unclear. Conclusions Splenectomy is a valid and safe diagnostic approach for adult HLH of unknown origin even though the patients with HLH were in severe condition with multiple organ dysfunction of some patients. The pathological diagnosis for resected spleen provided a clue for the further treatment.
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