Wang Hongluan, Xiong Lixia, Tang Weiping, Zhou Ying, Li Fei
Department of Hematology, The First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi 330006, China.
Medical College, Nanchang University, Nanchang, Jiangxi 330006, China.
Oncotarget. 2017 Jul 14;8(35):59977-59985. doi: 10.18632/oncotarget.19230. eCollection 2017 Aug 29.
As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable overlaps of symptoms with other types of HLH, thus resulting in higher incidence of misdiagnosis and mortality. In recent years, with the increasing awareness to this disease, the diagnosis and management of HLH have gained more and more attention, and improvements have been made accordingly. As a result, the survival of patients is greatly prolonged. However, there is still no consensus on the diagnostic criteria and treatment strategies due to lack of large samples or prospective clinical trials. In order to improve recognition and diagnosis, and provide guidance regarding the treatment of M-HLH, the Study Group in HLH Subtypes of the Histiocyte Society has developed consensus recommendations for the diagnosis and management of M-HLH in 2015. In the present article, we summarized and discussed some updated understandings in M-HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但可能危及生命的高炎症综合征，其临床特征为长期发热、肝脾肿大、血细胞减少、高甘油三酯血症、高铁蛋白血症以及骨髓、肝脏、脾脏或淋巴结中的噬血细胞现象。恶性肿瘤相关HLH（M-HLH）是获得性HLH的一种类型，通常与其他类型的HLH存在症状上的多种重叠，从而导致误诊率和死亡率较高。近年来，随着对该疾病认识的不断提高，HLH的诊断和治疗越来越受到关注，并相应取得了进展。因此，患者的生存期得到了显著延长。然而，由于缺乏大样本或前瞻性临床试验，在诊断标准和治疗策略方面仍未达成共识。为了提高对M-HLH的认识和诊断，并为其治疗提供指导，组织细胞协会HLH亚型研究组于2015年制定了M-HLH诊断和管理的共识建议。在本文中，我们总结并讨论了对M-HLH的一些最新认识。
