Hasselblom S, Linde A, Ridell B
Department of Medicine, Varberg Hospital, Varberg, Sweden.
J Intern Med. 2004 Feb;255(2):289-95. doi: 10.1046/j.0954-6820.2003.01249.x.
Haemophagocytic syndrome is a serious disorder, often related to Epstein-Barr virus (EBV) or other infectious agents. Frequently an underlying immune abnormality or a T-cell lymphoma is present. The combination of haemophagocytosis and Hodgkin's lymphoma seems to be rare. A 70-year-old female with rheumatoid arthritis was admitted with constitutional symptoms, persistent fever, pancytopenia, deranged liver enzymes, lymphadenopathy and splenomegaly. A fatal coagulopathy supervened. The clinical picture and the bone marrow findings indicated a haemophagocytic syndrome and a lymph node biopsy disclosed an EBV-positive Hodgkin's lymphoma. EBV serology pointed at viral reactivation and a high EBV DNA content was detected in serum by real-time quantitative PCR analysis (5.5 x 10(6) copies per mL). The case history is presented and the literature is reviewed.
噬血细胞综合征是一种严重的疾病，常与爱泼斯坦-巴尔病毒（EBV）或其他感染因子有关。通常存在潜在的免疫异常或T细胞淋巴瘤。噬血细胞现象与霍奇金淋巴瘤同时出现似乎较为罕见。一名70岁患有类风湿性关节炎的女性因全身症状、持续发热、全血细胞减少、肝功能酶紊乱、淋巴结病和脾肿大入院。随后出现了致命的凝血病。临床表现和骨髓检查结果提示为噬血细胞综合征，淋巴结活检显示为EBV阳性的霍奇金淋巴瘤。EBV血清学检查提示病毒再激活，通过实时定量PCR分析在血清中检测到高EBV DNA含量（每毫升5.5×10⁶拷贝）。本文介绍了该病例并复习了相关文献。
