肿瘤相关噬血细胞综合征的诊治

Diagnosis and treatment of malignancy-associated hemophagocytic lymphohistiocytosis

噬血细胞综合征是一种由各种诱因导致的细胞毒性T细胞、自然杀伤细胞和巨噬细胞异常激活,增殖失控,释放大量炎性因子引起的过度炎症反应综合征。肿瘤是导致继发性噬血细胞综合征的重要病因之一,发病率随着年龄的增长而增高。肿瘤相关噬血细胞综合征起病凶险,临床症状错综复杂,易致误诊、漏诊,死亡率高。近年来,随着对疾病认识的不断提高,肿瘤相关噬血细胞综合征的诊断和治疗策略不断更新,但仍缺乏大规模前瞻性临床研究指导疾病的规范化诊断和治疗。随着越来越多新药的涌现和临床研究的陆续开展,肿瘤相关噬血细胞综合征的诊断和治疗将不断完善,最终达到改善患者预后的目的。

Hemophagocytic lymphohistiocytosis(HLH)is a critical condition characterized by the undue activation of cytotoxic T lymphocytes,natural killer cells,and macrophages,resulting in excessive secretion of infammatory cytokines.Malignancy is an important trigger of secondary HLH,and the incidence of malignancy-associated HLH(M-HLH)increases with age.M-HLH is a life-threatening disease with an interdisciplinary complex of symptoms,high incidence of misdiagnosis and mortality.In recent years,with increasing awareness to this disease,the diagnosis and treatment of M-HLH have been renewed constantly.However,there is still no consensus on the diagnostic criteria and treatment strategies due to lack of large-scale prospective studies.With emerging drugs and ongoing clinical trials,it’s worth believing that the diagnosis and management of M-HLH will be optimized,which will ultimately improve prognosis.