17例淋巴瘤相关噬血细胞综合征的临床特点及疗效分析

Clinical characteristics and therapeutic effect of 17 cases of lymphoma-associated hemophagocytic syndrome

目的：总结淋巴瘤相关噬血细胞综合征（LAHS）的临床特点及疗效。方法分析17例 LAHS 患者的临床资料，总结其临床特点及疗效。结果17例患者均持续高热1周以上，15例出现脾大；17例血清铁蛋白均明显升高；16例出现全血细胞减少及高甘油三酯血症；14例有低纤维蛋白原血症；17例骨髓中均有噬血现象。17例中2例于化学治疗后接受异基因外周血造血干细胞移植术，已分别存活45个月及37个月；1例于化学治疗后接受自体外周血造血干细胞移植术，术后11个月死于感染；13例仅接受化学治疗者中1例存活15个月，其余12例均死亡，生存期为3～13个月。1例入院1周后即死亡者未接受治疗。结论LAHS 起病急、病情重，患者常有高热、脾大、血细胞减少、高血清铁蛋白及高甘油三酯血症，骨髓可见噬血现象。该类患者化学治疗疗效差，外周血造血干细胞移植术可能是较有效的治疗方法。

Objective To summarize the clinical characteristics and therapeutic effect of 1 7 cases of lymphoma-associated hemophagocytic syndrome （LAHS）.Methods The clinical data were analyzed to sum-marize the clinical features and therapeutic effect of 1 7 patients with LAHS.Results High-grade fever,high serum level of ferritin and hemophagocytosis were observed in 1 7 cases,cytopenia and hypertriglyceridemia in 1 6,splenomegaly in 1 5 and hypofibrinogenemia in 1 4.Two patients who received chemotherapy and then allo-geneic peripheral blood stem cell transplantation （allo-PBSCT）survived for 45 and 37 months.One case un-dergoing chemotherapy and then autologous peripheral blood stem cell transplantation （auto-PBSCT）died of in-fection at postoperative 1 5 months.Among 1 3 patients who received chemotherapy alone,only 1 case survived for 1 5 months after 8 cycles of chemotherapy and the other 1 2 died with a survival time ranging from 3 to 1 3 months.One case died one week after admission without any treatment.Conclusions LAHS is characterized as acute onset,severe condition,high-grade fever,hypertriglyceridemia,cytopenia,hemophagocytosis,high serum level of ferritin and hypertriglyceridemia.Chemotherapy yields relatively poor therapeutic effect,whereas allo-PBSCT may serve as an efficacious treatment of LAHS.