Department of Medicine and.
Department of Medicine and Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
Blood. 2015 May 7;125(19):2908-14. doi: 10.1182/blood-2015-01-551622. Epub 2015 Mar 10.
Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation. This syndrome can be caused by genetic mutations affecting cytotoxic function (familial HLH) or be secondary to infectious, rheumatologic, malignant, or metabolic conditions (acquired HLH). Prompt recognition is paramount and, without early treatment, this disorder is frequently fatal. Although HLH is well described in the pediatric population, less is known about the appropriate work-up and treatment in adults. Here, we review the clinical characteristics, diagnosis, and treatment of HLH in adults.
噬血细胞性淋巴组织细胞增生症（HLH）是一种破坏性的免疫激活失控疾病，其特征是存在临床和实验室证据表明存在极度炎症。这种综合征可能由影响细胞毒性功能的基因突变引起（家族性 HLH），也可能继发于感染、风湿病、恶性或代谢性疾病（获得性 HLH）。及时识别是至关重要的，如果不早期治疗，这种疾病通常是致命的。尽管 HLH 在儿科人群中已有很好的描述，但成人中适当的检查和治疗方法知之甚少。在这里，我们回顾了成人 HLH 的临床特征、诊断和治疗。
