噬血细胞综合征26例临床分析

Clinical analysis of 26 hemophagocytic syndrome cases

目的探讨噬血细胞综合征(HPS)的临床特点、诊断、治疗及临床预后因素。方法回顾性分析了26例噬血细胞综合征病因、临床特点、实验室检查、治疗与转归。结果发热、淋巴结肿大、肝脾肿大、皮肤瘀斑、黄染是最常见的临床表现。全血细胞减少(100.0%)、血清铁蛋白升高(61.5%)、血清乳酸脱氢酶增高(96.2%)、谷草转氨酶/谷丙转氨酶/总胆红素升高(73.1%)、凝血功能异常(65.4%)、甘油三酯增多(34.6%)是本组病例实验室特点;骨髓象:有核细胞增生大多活跃,均可见成熟组织细胞和噬血细胞。16例肿瘤相关性HPS,7例感染相关性HPS及3例原因不明HPS。结论 HPS可由多种原因引起,临床表现复杂多样,临床预后差,应积极寻找原发病因并对患者进行个体化治疗,有可能减少病死率。

Objective To investigate the clinical features, diagnosis, treatment and clinical prognostic factors of hemophagocytic syndrome （HPS）. Methods A retrospective study was carried out to analyze the pathogenesis, clinical characteristics, laboratory inspection, treatment and outcomes of 26 HPS cases. Results Common clinical characteristics were fever, lymphadenopathy, splenomegaly, hepatomegaly, petechia and jaundice. Laboratory data features included eytopenia （100.0%）, elevated ferritin （61.5%）, elevated lactate dehydrogenase （96.2%）, increased levels of total bilirubine tbil, aspartate transaminase and alanine aminotransferase （73.1%）, coagulation disorder （65.4%）, and high level of triglyeeride （34.6%）. The bone marrow was active, while hemophagocytosis and histiocytes could be seen in bone marrow. There were 16 cases with tumor associated HPS, 7 cases with infection-related HPS, and 3 case with HPS of unknown reason. Conclusion HPS can be caused by complicated reasons, and it has diverse clinical characteristics and poor prognosis. Therefore, finding pathogenesis and providing individualized treatment can possibly reduce the mortality.