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噬血细胞综合征(HPS),又称噬血细胞性淋巴组织细胞增生症,是一种由多种致病因素导致的单核-巨噬细胞系统反应性增生,释放大量的细胞因子,引起严重器官功能损害的疾病。肿瘤相关性HPS多见于淋巴瘤,又称淋巴瘤相关噬血细胞综合征(LAHS)。临床特征主要有发热、肝脾肿大、血细胞减少、凝血障碍、高铁蛋白血症、高三酰甘油血症、低纤维蛋白原血症、骨髓及其他脏器出现噬血细胞现象。LAHS病情凶险,病变进展快,预后差。化学疗法联合大剂量皮质类固醇激素及免疫抑制治疗是该病的主要治疗方案,但疗效差,患者多在短期内死亡。该文就LAHS的发病机制、临床特征、诊断、治疗等方面的研究进展加以综述,旨在提高对LAHS的认识,指导临床治疗。
Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis, is a system of monocyte-macrophage reactive hyperplasia caused by a variety of causative factors that release large quantities of cytokines and cause serious organ problems Functional impairment of the disease. Tumor-related HPS more common in lymphoma, also known as lymphoma-associated hemophagocytic syndrome (LAHS). The main clinical features are fever, hepatosplenomegaly, cytopenia, coagulopathy, ferritin, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis in bone marrow and other organs. LAHS disease is dangerous, rapid progression of the disease, the prognosis is poor. Chemotherapy combined with high-dose corticosteroids and immunosuppressive therapy is the main treatment of the disease, but the curative effect is poor, more patients died in the short term. This article reviews the research progress on the pathogenesis, clinical features, diagnosis and treatment of LAHS so as to improve the understanding of LAHS and guide the clinical treatment.