成人噬血细胞综合征23例临床特征分析

Retrospective analysis of clinical features in 23 cases with adults′hemophagocytic syndrome

目的分析成人噬血细胞综合征(HPS)的临床特征,对比HLH-2004诊断标准,以提高对本病的认识,早期诊断,减少误诊,提高存活率。方法回顾分析近5年收治的23例因肝功能异常入院最终确诊为噬血细胞综合征患者的病因、临床症状、体征、实验室检查结果及转归。结果 23例患者原发病分析显示,恶性淋巴瘤9例,感染8例,风湿性疾病5例,其他原因1例。主要临床表现以持续发热(95.7%)、脾脏肿大(100.0%)及肝脏肿大(82.6%)为突出表现,其他表现为淋巴结肿大(73.9%)、黄疸(73.9%)、呼吸系统症状(52.2%)、多浆膜腔积液(56.5%)、中枢神经系统症状(47.8%)、皮疹(34.8%)、出血(21.7%)及肾功能损害(30.4%)。实验室检查以肝功能损害最为突出,主要是LDH、AST升高(100.0%),血细胞减少(两种血细胞下降占30.4%、全血血细胞下降占69.6%),纤维蛋白原(Fg)下降(82.6%),PT延长(47.8%)。死亡组患者的LDH和AST水平明显高于存活组(t=4.509、3.339,P=0.003),而死亡组患者的血小板计数和Fg水平明显低于存活组(t=7.892、3.561,P=0.002)。首次骨髓检查有噬血细胞现象14例,第2次或多次骨髓检查有噬血细胞现象9例。23例经治HPS患者12周总体生存率为43.5%。结论 HPS可由多种病因引起,临床表现多样,病死率高。LDH和AST升高,PLT和Fg降低是疾病的不良预后因素。骨髓的多次检查有助于及时诊断。

Objective To investigate the clinical features of hemophagocytic syndrome （HPS）and to improve its recognition for leading to early diagnosis and increasing survival rate,compased with HLH-2004 diagnostic criteria. Methods A retrospective study was carried out to analyze the etiology,clinical characteristic,laboratory findings and outcomes in 23 patients with HPS. Results Among theses 23 cases,the initiating causes were as follows：9 cases with lymphoma （39.1% ）,8 cases with infection （34.8% ）,5 cases with rheumatoid diseases （21 .7% ）and 1 case with unknown etiology （4.8% ）. HPS was characterized typically by prolonged fever （95.7% ）,splenomegaly （100.0% ）,hepatomegaly （82.6% ） and other features including lymph adenopathy （73.9% ）,respiratory symptoms （52.2% ）,hydrops of multiple serous cavity （56.5% ）,jaundice （73.9% ）,central nervous system involvement （47.8% ）,skin rash （34.8% ）,alimentary tract hemorrhage （21 .7% ）and injury of kidney （30.4% ）. According to laboratory data,liver dysfunction was pointed out to be the most prominent manifestation,characterized by elevated LDH and AST （100% ）. The peripheral cytopenia in two （30.4% ）or three （69.6% ）lineages,decreased fibrinogen （82.6% ）and prolonged prothrombin time （47.8% ）were observed. LDH and AST level were significantly higher in death group than those in survival group （t= 4.509,P= 0.000;t= 3.339,P= 0.003）. Nevertheless,the platelet count and fibrinogen level were significantly lower in death group than those in survival group （t= 7.892,P= 0.000;t= 3.561,P= 0.002）. In 60.9% of HPS cases,hemophagocytosis was observed in bone marrow at the first bone marrow puncture,while in 39.1% of HPS cases,hemophagocytosis was observed in bone marrow at the following repeated bone marrow puncture. The 12-week survival rate was 43.5% in 23 cases with treatment. Conclusion HPS can be induced by various etiologies and manifested by different symptoms with high mortality. LDH,AST,platelet count and fibrinogen level might be the prognostic predictors of outcome. Repeated bone marrow examinations are needed to help attaining early diagnosis.