成人噬血细胞性淋巴组织细胞增生症的临床特征及预后分析

Clinical features of 41 cases of adult hemophagocytic lymphohistiocytosis and prognosis analysis

目的分析成人噬血细胞性淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis，HLH）的临床特征，探讨影响其预后的危险因素。方法回顾性研究复旦大学附属华山医院2006—01～2012—05收治的41例HLH患者临床、实验室检查等资料，采用多因素分析法对预后相关危险因素进行研究。结果发热、关节肌肉症状、肝脾大是HLH最为常见的临床特征，皮疹、腹泻则相对少见；而实验室检查方面最具特征性的表现为全血细胞减少、高甘油三酯血症、低纤维蛋白原血症、铁蛋白显著升高，以及骨髓噬血细胞现象；多浆膜腔积液、伴发感染和低血小板计数是HLH预后不良的独立危险因素，其中血小板计数〈5×10^9／L是最强危险因素（OR：71．5，95％CI=2．48～2061）。结论成人HLH病情严重，预后凶险，病死率极高，多浆膜腔积液、伴发感染和低血小板计数（〈5×10^9／L）是影响其预后的主要因素。

Objective To evaluate the clinical features of hemophagocytic lymphohistiocytosis （HLH） in adult and to discuss the risk factors affecting the prognosis. Methods Forty - one patients with HLH who admitted to Huashan Hospital, Fudan University from January 2006 to May 2012 were reviewed retrospectively. Clinical features and laboratory information were collected. Multivariate analysis was conducted to analyze the risk factors. Results Fever, articular and muscular symptoms and hepatosplenomegaly were the most common clinical features of HLH. Rash and diarrhea were relatively rare. The most common laboratory findings were pancytopenia, hypertriglyceridemia, hypofibrinogenemia, significant elevation in serum ferritin levels and hemophagocytosis in bone marrow. Multiple serous cavity effusion, infection and lower platelet count were independent risk factors of poor prognosis. Platelet count less than 5 x 109/L was the most powerful risk factor（OR = 71.5, 95% CI = 2. 48 ~ 2061 ） Conclusion Adult HLH is a serious disease with an extremely poor outcome which leading to high mortality. Multiple serous cavity effusion, infection and lower platelet count less than 5 x 109/L are the main factors which affect the prognosis.