Dierickx D, Cortoos A, Vanderschueren S
Department of Haematology, University Hospitals Leuven, Belgium.
Acta Clin Belg. 2011 Nov-Dec;66(6):448-52. doi: 10.2143/ACB.66.6.2062613.
The haemophagocytic syndrome or haemophagocytic lymphohistiocytosis (HLH) is a syndrome encompassing a heterogeneous group of disorders characterized by a persistent activation of benign macrophages, leading to uncontrolled secretion of cytokines and phagocytosis of blood cells. The syndrome can be primary due to mutations in different genes crucially involved in lymphocyte cytotoxicity and secondary in association with infectious, autoimmune or malignant disorders. In most cases HLH displays an aggressive disease course with a high fatality rate without treatment. Early recognition of the syndrome and prompt initiation of appropriate treatment, in most cases consisting of immunochemotherapy, are mandatory to ensure long-term survival.
噬血细胞综合征或噬血细胞性淋巴组织细胞增生症（HLH）是一种综合征，涵盖一组异质性疾病，其特征是良性巨噬细胞持续激活，导致细胞因子不受控制地分泌以及血细胞被吞噬。该综合征可以是原发性的，由与淋巴细胞细胞毒性密切相关的不同基因突变引起，也可以是继发性的，与感染性、自身免疫性或恶性疾病相关。在大多数情况下，HLH表现出侵袭性病程，未经治疗死亡率很高。早期识别该综合征并及时开始适当治疗（在大多数情况下包括免疫化疗）对于确保长期生存至关重要。
