19例成人噬血细胞综合征临床分析

Clinical analysis of 19 adult patients with hemophagocytic syndrome

目的：探讨成人噬血细胞综合征（HPS）的临床特点,旨在进一步提高对该病的认识。方法：回顾分析19例成人HPS的临床资料。结果：19例患者中男11例,女8例;中位发病年龄34（18~61）岁。考虑肿瘤相关5例（26.3%）,感染相关5例（26.3%）,结缔组织病4例（21.1%）,原因不明5例（26.3%）。19例患者（100%）均有高热,14例（73.7%）表现为肝脾肿大,5例（26.3%）表现为多发浅表淋巴结肿大,3例（15.8%）出现皮疹,3例（15.8%）出现神经系统症状。19例患者均出现血清铁蛋白及血清乳酸脱氢酶升高,18例（94.7%）可见血细胞减少,14例（73.7%）血清甘油三酯水平升高,5例（26.3%）出现低纤维蛋白原血症,12例（63.2%）出现肝功能异常。4例（21.1%）EB病毒DNA滴度升高。全部患者于骨髓中发现吞噬细胞现象。全组患者8例存活,7例死亡,4例失访。19例患者的生存时间为0.3~39.8个月（中位生存时间为4.7个月）,病死率36.8%。结论：HPS是一种少见疾病,其病因复杂,进展迅速,病死率高,且发病机制尚未明确,提高对HPS的认识,做出早期诊断和及时的治疗至关重要。

Objective： To investigate the clinical features of adult patients with hemophagocytic syndrome （HPS） and to improve recognition of HPS. Method： The clinical and experimental data of 19 adult patients with HPS were collected. Result： Of 19 patients, male in 11 cases, women in 8 cases, the median onset age was 34 （ 18 to 61）. The initiating causes were as follows：5 cases （26.3%） with tumor,5 cases （26.3%） with infection,4 cases （21.1%） with rheumatoid diseases and 5 cases （26.3%） with unknown etiology. HPS was characterized typically by fever （ 100 % ）, splenomegaly （73.7% ）, hepatomegaly （26.3 %） and other features including lymph adenopathy （26.3%） ,skin rash （15.8%）,and central nervous system involvement （15.8%）. According to laboratory data, two or three cytopenia （94.7 % ）, heperferritinemia （ 100 %）, elevated lactate dehydrogenase （100 %）, hypertriglyceridemia （73. 7%）, hypofibrinogenemia （26. 3%） and liver dysfunction （ 63. 2 %） were observed. Four cases （21.1%） were found to be infected with EB virus. Hemophagocytosis was observed in bone marrow in all patients. Eight cases were survival during the follow-up and 7 cases were dead,4 cases lost follow-up. The median survival time was 4.7 months （range 0.3 to 39.8 months）. Conclusion： HPS is an uncommon fatal disease and it has very complex clinical manifestations, aggressive course, poor prognosis and high death rate. A high index of suspicion is the critical factor for early diagnosis,and early treatment with immunosuppressant is warranted.