摘要
目的总结继发性噬血细胞性淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)患者的临床特点。方法回顾性分析北京协和医院2007年1月至2012年6月收治的127例继发性HLH患者的潜在疾病、临床特征、实验室检查结果及预后。结果 HLH患者的潜在疾病包括病毒感染(26例,20.5%)、结核感染(4例,3.1%)、自身免疫病(16例,12.6%)、血液系统恶性肿瘤(48例,37.8%)和原因不明者(33例,26.0%)。常见临床表现有发热(97.6%)、肝大(53.5%)、脾大(79.5%)、淋巴结肿大(55.1%)、消化道出血(13.4%)、肾损害(29.9%)及中枢神经系统损害(12.6%)。实验室检查可见血细胞减少(98.4%)、肝功能异常(97.6%)、低纤维蛋白原血症(67.5%)、高甘油三酯血症(85.2%)、高铁蛋白血症(97.5%)和骨髓涂片中的噬血现象(91.3%)。44例治愈的患者中,37例长期随访仍持续缓解。83例因潜在疾病进展、多器官功能衰竭或严重感染死亡。结论继发性HLH可见于多种病因,临床表现多样,患者病情往往较重、病死率高。起病年龄大、男性、脾大及合并弥散性血管内凝血(DIC)可能会影响HLH患者预后。
Objective To summarize the clinical features of patients with hemophagocytic lymphohistiocytosis(HLH)by analyzing the clinical features of 127 patients.Methods The underlying diseases,clinical characteristics,laboratory findings,responses to therapy as well as outcomes of the 127 patients with HLH were analyzed retrospectively.Results The underlying diseases included virus infection(n =26),tuberculosis infection(n =4),rheumatic diseases(n =16),heamatologic malignancies(n=48),and no causes could be identified(n=33).HLH was clinically characterized by high fever(97.6%),hepatomegaly(53.5%),splenomegaly(79.5%),lymphadenopathy(55.1%),gastrointestinal hemorrhage(13.4%),renal(29.9%)and central nervous system(12.6%)involvement.The major laboratory findings included cytopenia(98.4%),liver dysfunction(97.6%),hypofibrinogenemia(67.5%),hypertriglyceridemia(85.2%),serum ferritin(97.5%),and hemophagocytosis in bone marrow(91.3%).Forty-four patients recovered after treatment,and 37 patients archieved long-term remission during the follow-up.Eighty-three patients died because of the progression of the underlying diseases,multiple organ failure and severe infection.Conclusion There are various underlying diseases for HLH and there are many clinical characteristic manifestations for HLH.HLH is always a critical situation with high mortality.Late disease onset,male gender,splenomegaly and DIC may affect the outcome of HLH patients.
出处
《北京医学》
CAS
2013年第5期360-362,366,共4页
Beijing Medical Journal
关键词
噬血细胞性淋巴组织细胞增生症
潜在疾病
临床表现
预后
危险因素
Hemophagocytic lymphohistiocytosis(HLH)Underlying diseases Clinical manifestation Prognosis Risk factor