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噬血细胞性淋巴组织细胞增生症 (HLH)是一组以淋巴细胞、巨噬细胞非恶性增生伴噬血细胞增多引起多脏器浸润及全血细胞减少为特征的疾病。HLH分为两种亚型 :原发性HLH和继发性HLH。其临床表现相同 ,主要有持续发热、肝脾肿大以及神经系统受累的症状和体征。实验室检查可见全血细胞减少、高脂血症、低纤维蛋白原血症、血浆铁蛋白增多、肝功异常 ,骨髓、肝、脾、淋巴结活检可见明显的组织细胞增生伴吞噬血细胞现象。目前HLH的治疗仍采用化疗与免疫抑制治疗相结合的方法 ,骨髓移植更为有效。HLH预后差 ,病死率高。
Hemophagocytic lymphohistiocytosis (HLH) is a group of diseases characterized by non-malignant proliferation of lymphocytes, macrophages with increased hemophagocytosis causing multiple organ infiltration and pancytopenia. HLH is divided into two subtypes: primary HLH and secondary HLH. The clinical manifestations of the same, there are persistent fever, hepatosplenomegaly and nervous system involvement symptoms and signs. Laboratory tests showed pancytopenia, hyperlipidemia, hypofibrinogenemia, increased plasma ferritin, abnormal liver function, bone marrow, liver, spleen, lymph node biopsy showed obvious cell proliferation with phagocytic phenomenon. The current treatment of HLH is still using a combination of chemotherapy and immunosuppressive therapy, bone marrow transplantation is more effective. HLH poor prognosis, high mortality.