目的：分析继发性噬血细胞综合征（HPS）患者的实验室指标及临床特点，以提高对该病的认识。方法对2005年6月～2015年6月在广西肿瘤防治研究所治疗的32例继发性 HPS 患者的病因、临床表现、实验室特征、治疗及预后等进行分析。结果32例患者中，血液系统肿瘤17例，单纯感染13例，自身免疫性疾病2例。临床上以发热（100％）、肝脾（87．5％）及淋巴结（43．8％）肿大为主要表现。实验室检查以血细胞减少（100％）、铁蛋白升高（96．9％）、自然杀伤细胞活性降低或缺失（96．9％）为主，纤维蛋白原降低（68．8％），甘油三酯升高（56．3％），81．3％的患者骨髓涂片检查可见噬血细胞现象。结论继发性HPS 可由多种病因引起，临床表现多样，对可疑患者及时进行相关实验室检查有助于早期明确诊断；合并多脏器衰竭、DIC 的患者预后不良。%Objective To summarize the clinical features and laboratory data of 32 patients with secondary he‐mophagocytic syndrome(HPS) . Methods 32 patients of HPS were continuously collected from 2005 to 2015 at our hospital .Their underlying diseases ,clinical features ,laboratory data ,therapy and prognosis were analyzed . Results The gender ratio was roughly equal .The causes were hematologic neoplasm (n=17) ,infection(n=13) , autoimmune diseases (AID ,n=2) .HPS was characterized by high fever (100% ) ,hypersplenomegaly (87 .5% )and lymphadenopathy(43 .8% ) .Laboratory data showed cytopenia(100% ) ,serum ferritin elevation (96 .9% ) ,low NK‐cell activity(96 .9% ) ,hypofibrinogenemia(68 .8% ) ,hypertriglyceridemia(56 .3% ) and hemophagocytosis in bone marrow(81 .3% ) .30 patients were treated with HLH‐2004 .Pulse‐dose corticosteroid therapy was used in 2 AID patients .And 14 patients died and 2 withdrew treatment because of exacerbation .8 progressed into MODS and an‐other 4 had complications of DIC .Neoplasm(58 .8% ) had the highest mortality in secondary HPS . Conclusion Secondary HPS can be induced by multiple etiologies and manifested by different symptoms .Relevant laboratory tests for suspected patients may aid an early diagnosis .Presence of MODS or DIC in HPS is possibly correlated with a poor prognosis and a high mortality .
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