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首页> 外文期刊>Orphanet journal of rare diseases >Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China
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Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China

机译：成人吞噬细胞综合征患者的临床特征和预后因素：一项回顾性研究，旨在提高中国单中心对疾病的认识

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Background Hemophagocytic lymphohistiocytosis (HLH) is a relatively rare but life-threatening disease with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality. Methods To improve the recognition as well as understanding of this disorder, we analyzed clinical characteristics and prognostic factors from 85 adult patients diagnosed with HLH in our hospital from April 2005 to June 2014. Results Patients with HLH displayed variable clinical markers across a wide spectrum. These included fever and hyperferritinemia (100%), elevated lactate dehydrogenase (LDH) (98.8%), two or three cytopenia (92.2%), splenomegaly (72.9%), hypofibrinogenemia (69.4%), hypertriglyceridemia (64.7%), hemophagocytosis (51.7%), and hepatomegaly (24.7%). Patients with active Epstien-Barr Virus (EBV) infection had a median overall survival (OS) of 65 days. Those displaying malignancy had very poor survival (median OS: 40 days). However, patients in rheumatic and non-EBV infection groups had relatively superior prognosis (not reached). Univariate analysis showed that Fibrinogen (Fbg) <1.5 g/L, platelet number (PLT)?<40 × 109/L and LDH ≥1000 U/L were factors that negatively affected survival (P = 0.004, 0.000, 0.002). Multivariate analysis showed that PLT <40 × 109/L was the independent adverse factor (HR = 0.350, 95% CI: 0.145-0.844, P = 0.019). Conclusions HLH had very complex clinical manifestations and high death rate. Patients with active EBV infection, malignancy, Fbg <1.5 g/L, PLT <40 × 109/L and LDH ≥1000 U/L had high risk of death as well as inferior survival, and these patients require systemic targeted treatments as early as possible.
机译：背景吞噬性淋巴细胞组织细胞增生症（HLH）是一种相对罕见但危及生命的疾病，具有令人困惑的临床表现，健康状况迅速恶化，高发病率和高死亡率。方法为提高对这种疾病的认识和了解，我们分析了我院自2005年4月至2014年6月诊断为HLH的成年患者的临床特征和预后因素。结果HLH患者在广泛的范围内显示出可变的临床标志物，其临床特征和预后因素来自于2005年4月至2014年6月。这些包括发烧和高铁蛋白血症（100％），乳酸脱氢酶（LDH）升高（98.8％），两到三个血细胞减少症（92.2％），脾肿大（72.9％），低纤维蛋白原血症（69.4％），高甘油三酸酯血症（64.7％），吞噬作用（ 51.7％）和肝肿大（24.7％）。活动性EB病毒感染的患者中位总生存期（OS）为65天。那些表现出恶性肿瘤的患者生存率很低（中位OS：40天）。但是，风湿性和非EBV感染组的患者预后相对较好（未达到）。单因素分析表明，纤维蛋白原（Fbg）<1.5 g / L，血小板数（PLT）？<40×109 / L和LDH≥1000U / L是对存活率产生负面影响的因素（P = 0.004，0.000，0.002）。多因素分析表明，PLT <40×109 / L是独立的不利因素（HR = 0.350，95％CI：0.145-0.844，P = 0.019）。结论HLH临床表现非常复杂，死亡率高。活动性EBV感染，恶性肿瘤，Fbg <1.5 g / L，PLT <40×109 / L和LDH≥1000U / L的患者有很高的死亡风险，并且生存期较差，这些患者需要尽早进行全身靶向治疗可能。

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