pet-ct在噬血细胞综合征病因诊断中的应用-第三军医大学学报.docVIP

11例淋巴瘤相关噬血细胞综合征的临床特点及疗效分析 李旭东 王东宁 何易 胡元 王文文 黄仁魏* （中山大学附属第三医院血液科，广东，广州，510630） 【摘要】 目的：分析11例淋巴瘤相关噬血细胞综合征（lymphoma-associated hemophagocytic syndrome,LAHS)的临床特点并观察临床疗效。方法：2009年3月-2013年10月在我院血液科诊断的11例LAHS患者，分析其临床和实验室检查特点并观察临床疗效。结果：11例患者均有高热、高铁蛋白血症及骨髓中出现噬血现象，10例有全血细胞减少及高甘油三酯血症，9例有脾大及低纤维蛋白原血症。11例患者中2例经化疗后接受异基因造血干细胞移植术，已存活45个月及37个月；1例接受8疗程化疗者已存活15个月；1例化疗后行自体造血干细胞移植术，术后11个月死于感染；6例仅接受化疗者均死亡，生存期4-14个月；另1例未接受治疗即死亡。结论：LAHS具有起病急、病情重、高热、脾大、血细胞减少、噬血现象、高血清铁蛋白及高甘油三酯的临床特点，该类患者诊断困难，化疗疗效差，异基因造血干细胞移植可能是最有效的治疗方法。 【关键词】 噬血细胞综合征；淋巴瘤；临床特点；疗效 Clinical characteristics and therapeutic effect of 11 cases of lymphoma-associated hemophagocytic syndrome Li Xudong, Wang Dongning, He Yi , Hu Yuan, Wang Wenwen, Huang Renwei* (Department of Hematology, The Third Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510630, China) [ABSTRACT] Objective: To observe the clinical characteristics and therapeutic effect of 11 cases of lymphoma-associated hemophagocytic syndrome(LAHS). Methods: The clinical and laboratory characteristics were analysised and therapeutic effect was observed in 11 cases of LAHS between March 2009 and October 2013. Results: High-grade fever, high serum ferritin level and hemophagocytosis were observed in 11 cases, cytopenia and hypertriglyceridemia in 10 cases, splenomegaly and hypofibrinogenemia in 9 cases. 2 patients have survived separately for 45 and 37 months after chemotherapy and allogeneic peripheral blood stem cell transplantation(allo-PBSCT), and 1 patient has survived for 15 months after 8 cycles of chemotherapy. 1 case died of infection 15 months after chemotherapy and autologous peripheral blood stem cell transplantation(auto-PBSCT). 6 cases who received chemotherapy only were all dead after 4-14 months, and 1 case died soon without therapy. Conclusions: Clinical characteristics of LAHS are urgent and serious condition, high-grade fever, hypertriglyceridemia, cytopenia, hemophagocytosis, high serum ferritin level and hypertriglyceridemia. Allo-PBSCT maybe t