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与病毒有关的吞噬血细胞综合征(Virus-asso-ciated Hemophagocytic Syndrome简称VAHS)并非恶性疾病,其骨髓标本中显示组织细胞过度增生伴明显吞噬血细胞现象。作者将19树本病患者分为两组:第1组不伴其他明显疾病,兄弟姐妹间无与患者同样病史。第2组14例其中一例而全身性红斑性狼疮,其余13例均为接受肾移植的患者,且在罗致VA-HS前均接受过硫唑嘌呤与强的松治疗及13例曾作过脾切除。该14例用免疫抑制剂治疗开始后到出现VA-HS为2周至88个月(平均8个月)。在临床方面,19例均有严重的全身症状及高热,10例有肝肿大,5例脾肿大(除外13例在病前已作过脾切除)。16例作肝功检查结果14例异常,13例患者有血液凝固异常,14例有免疫抑制。病毒研究:采用补体结合试验、免疫萤光测定和细胞内含有病毒的细胞包涵体检查,在19例中除4例
Virus -associated Hemophagocytic Syndrome (VAHS) is not a malignant disease. His bone marrow specimens showed hyperplasia of histiocytes and significant phagocytosis of blood cells. The authors divided 19 tree patients into two groups: group 1 without any other obvious disease and no relationship between brothers and sisters. In group 2, 14 cases were diagnosed as systemic lupus erythematosus (SLE), and the remaining 13 cases were all recipients of renal transplantation. Both patients received prior azathioprine and prednisone treatment before VA-HS and 13 cases of splenomegaly resection. The onset of VA-HS in 14 of the 14 patients treated with immunosuppressive agents was from 2 weeks to 88 months (mean, 8 months). In the clinical field, 19 patients had severe systemic symptoms and hyperthermia, 10 had hepatomegaly and 5 had splenomegaly (except 13 patients who had been splenectomized before the disease). Of the 16 cases, 14 were abnormal in liver function test, 13 were abnormal in blood coagulation and 14 were immunosuppressed. Virus research: using complement fixation test, immunofluorescence and intracellular virus containing cell inclusion examination, in 19 cases, except 4 cases