成人噬血细胞淋巴组织增生症30例临床分析

目的：探讨噬血细胞淋巴组织增生症（HLH）的病因及临床特点，提高对HLH的认识。方法：回顾性分析30例噬血细胞淋巴组织增生症患者的临床资料。结果：符合Imashuku继发性HLH修订标准的27例患者中，病因不明HLH8例，占30％，其中6例病情危重。肿瘤相关HLH10例，占37％，其中5例T／NK细胞淋巴瘤4例死亡。各种感染（包括曲霉菌、伤寒杆菌、EB病毒等）相关HLH5例，占19％，1例EB病毒感染者死亡。自身免疫性疾病相关HLH4例，占14％，免疫抑制治疗效果佳。噬血细胞占骨髓有核细胞〈2％组与≥2％组在病因构成、血小板数量等方面无显著差异。结论：成人HLH多为继发性，应尽可能明确病因，采取针对性治疗。危重患者宜尽早采用免疫化疗或尝试异基因造血干细胞移植。噬血细胞占骨髓有核细胞比例的高低不影响HLH的诊断。

Clinical analysis of 30 cases of adult hemophagocytic lymphohistiocytosis

Objective:To investigate causes and analyze clinical features of adult hemophagocytic lymphohistiocytosis(HLH).Methods:Thirty cases of adult HLH were retrospectively analyzed.Results:In 21 patients consistent with Imashuku's modified HLH criteria,the causes of 8 patients(30%) were unknown,and 6 patient's condition were severe.In 10 malignancy-associated HLH patients(37%),four in five T/NK cell lymphomas patients died.There were 5 infection-associated HLH patients(19%),one EBV-HLH patient died.Four autoimmune disease-associated HLH patients(14%) with immunosuppressive therapy obtained good effect.No satistically significant difference was found between the group with hemophagocyte in bone marrow less than 2% and the one more than 2% in cause,platelet level and others.Conclusion:Adult hemophagocytic lymphohistiocytosis is usually secondary,causes should be investigated in order to take suitable measure.The severe patients would be treated with immunochemotherapy as early as possible or try allogeneic hematopoietic stem cell transplantation.Hemophagocyte proportion in bone marrow does not influence diagnose of HLH.