噬血细胞性淋巴组织细胞增生症相关病因研究进展

Research Progress in the Etiology of Hemophagocytic Lymphohistiocytosis

噬血细胞性淋巴组织细胞增生症(HLH)是一种血液病的急危重症,其具有起病急、进展快、预后较差等临床特点。导致HLH的原因众多,遗传因素、感染性疾病、肿瘤、自身免疫相关性疾病等均可为本病的致病因素。感染相关性HLH和自身免疫疾病相关性HLH以基础疾病治疗最为关键,预后相对较好,而原发性HLH和肿瘤相关性HLH最有效的治疗方法是造血干细胞移植,但预后欠佳。

Hemophagocytic lymphohsitiocytosis （ HLH ） is a serious disease among blood diseases. The clinical features of it include acute onset, rapid progress and poor prognosis. Many etiologies can cause HLH, including genetic factors, infection, malignant tumor, autoimmune related diseases and other diseases. In the infection related HLH and autoimmune-associated HLH, basic disease treatment is the key and the prognosis is usually better. In the primary HLH and tumor related HLH, the most effective treatment is hematopoietic stem cell transplantation, but the prognosis is often poor.