Esteban Ysabella M, de Jong Jill L O, Tesher Melissa S
Pediatr Ann. 2017 Aug 1;46(8):e309-e313. doi: 10.3928/19382359-20170717-01.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by a dysregulated hyperinflammatory response associated with aberrant activation of lymphocytes and macrophages that results in hypercytokinemia. It is classically divided into two types: (1) primary or familial HLH and (2) secondary HLH. Familial HLH is generally an autosomal recessive condition, whereas secondary HLH is usually associated with infectious diseases, autoinflammatory and autoimmune diseases (where it is more commonly known as macrophage activation syndrome), malignancy, immunosuppression, hematopoietic stem cell transplantation, organ transplantation, HIV infection, and metabolic diseases. Although its clinical presentation is often similar to bacterial sepsis or systemic inflammatory response syndrome, HLH can be life-threatening. As such, it is imperative to recognize and diagnose HLH in a timely manner to optimize care. [Pediatr Ann. 2017;46(8):e309-e313.].
噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的综合征，其特征为与淋巴细胞和巨噬细胞异常激活相关的炎症反应失调，导致细胞因子血症。它通常分为两种类型：（1）原发性或家族性HLH和（2）继发性HLH。家族性HLH一般为常染色体隐性疾病，而继发性HLH通常与传染病、自身炎症性和自身免疫性疾病（在这些疾病中它通常被称为巨噬细胞活化综合征）、恶性肿瘤、免疫抑制、造血干细胞移植、器官移植、HIV感染和代谢性疾病相关。尽管其临床表现通常与细菌性败血症或全身炎症反应综合征相似，但HLH可能危及生命。因此，及时识别和诊断HLH以优化治疗至关重要。[《儿科年鉴》。2017年；46（8）：e309 - e313。]
