Kang Jing, Wang Shi-Xuan, Li Fei
Center of Hematology, The First Affiliated Hospital of Nanchang University, Institute of Hematology, Academy of Clinical Medicine of Jiangxi Province, Institute of Lymphoma and Myeloma in Nanchang University, Nanchang 330006, Jiangxi Province, China.
Center of Hematology, The First Affiliated Hospital of Nanchang University, Institute of Hematology, Academy of Clinical Medicine of Jiangxi Province, Institute of Lymphoma and Myeloma in Nanchang University, Nanchang 330006, Jiangxi Province, China,E-mail:
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2022 Jun;30(3):959-964. doi: 10.19746/j.cnki.issn.1009-2137.2022.03.048.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease caused by uncontrolled proliferation of activated macrophage, and secreting high amounts of inflammatory cytokines which lead to multi-organ dysfunction syndrome. HLH patients often show different clinical characteristics during the disease was progressed, in which coagulopathy were the most common, including thrombocytopenia and hypofibrinogenemia, those are the major cause of death in patients, and the clinicians should increase awareness of the mechanisms, clinical characteristics, prognosis and treatment. In this review, the above problems are briefly summarized, to deepen understanding of the HLH related coagulation dysfunctions, and early identification and treatment to reduce mortality, so as to provide more opportunities for HLH patients to recieve subsequent treatment.
噬血细胞性淋巴组织细胞增生症（HLH）是一种由活化巨噬细胞不受控制地增殖引起的危及生命的疾病，活化巨噬细胞分泌大量炎性细胞因子，导致多器官功能障碍综合征。HLH患者在疾病进展过程中常表现出不同的临床特征，其中凝血病最为常见，包括血小板减少和纤维蛋白原血症，这些是患者死亡的主要原因，临床医生应提高对其机制、临床特征、预后及治疗的认识。在本综述中，对上述问题进行了简要总结，以加深对HLH相关凝血功能障碍的理解，并进行早期识别和治疗以降低死亡率，从而为HLH患者提供更多接受后续治疗的机会。
