Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis
Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis
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DOI：
10.1002/ajh.23911
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发表时间：
2015-03-01
影响因子：
12.8
通讯作者：
Eby, Charles S.
中科院分区：
文献类型：
--
作者：
Otrock, Zaher K.;Eby, Charles S.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的临床综合征，其特征是单核吞噬细胞系统的激活。成人HLH的诊断具有挑战性，不仅因为大多数报告的数据来自儿科患者，而且因为HLH发生在许多疾病实体中。本研究报告了2003年至2014年在一家医疗中心管理的大型队列中成人HLH的临床和实验室检查结果以及预后因素。73例患者符合HLH-2004诊断标准。中位年龄为51岁（范围：18-82岁）; 41例（56.2%）为男性。>85%的患者表现为发热、血细胞减少和铁蛋白升高。HLH的可能原因如下：30例（41.1%）感染，21例（28.8%）恶性肿瘤，5例（6.8%）归因于自身免疫性疾病，1例（1.4%）原发性免疫缺陷，2例（2.7%）实体器官移植后，13例（17.8%）特发性。中位总生存期为7.67个月。与非恶性肿瘤相关的HLH患者相比，恶性肿瘤相关的HLH患者的生存率明显较差（中位总生存期分别为1.13个月和46.53个月; P <50，000 μ g/L与30天死亡率相关。诊断为HLH后的生存率很低，尤其是恶性相关HLH患者。成人HLH登记的发展将提高我们对这种综合征的理解，验证诊断标准，并帮助制定有效的治疗策略。Am. J. Hematol. 90：220-224，2015. (c)2014 Wiley Periodicals，Inc.
Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by the activation of the mononuclear phagocytic system. The diagnosis of HLH in adults is challenging not only because the majority of the reported data are from pediatric patients, but also because HLH occurs in many disease entities. This study reports the clinical and laboratory findings and prognostic factors of adult HLH in a large cohort managed at a single medical center from 2003 to 2014. Seventy-three patients met the HLH-2004 diagnostic criteria. The median age was 51 years (range, 18-82 years); 41 (56.2%) were male. Patients manifested fever, cytopenias, and elevated ferritin in >85% of cases. Likely causes of HLH were as follows: 30 (41.1%) infections, 21 (28.8%) malignancies, 5 (6.8%) attributed to autoimmune disorders, 1 (1.4%) primary immunodeficiency, 2 (2.7%) post solid organ transplantation, and 13 (17.8%) idiopathic. The median overall survival was 7.67 months. Patients with malignancy-associated HLH had a markedly worse survival compared with patients with non-malignancy-associated HLH (median overall survival 1.13 vs. 46.53 months, respectively; P50,000 mu g/L correlated with 30-day mortality. Survival after a diagnosis of HLH is dismal, especially among those with malignancy-associated HLH. The development of a registry for adults with HLH would improve our understanding of this syndrome, validate diagnostic criteria, and help develop effective treatment strategies. Am. J. Hematol. 90:220-224, 2015. (c) 2014 Wiley Periodicals, Inc.