Wong K F, Chan J K, Lo E S, Wong C S
Department of Pathology, Queen Elizabeth Hospital, Hong Kong.
Hum Pathol. 1996 Nov;27(11):1239-42. doi: 10.1016/s0046-8177(96)90321-4.
The herpes group of viruses, particularly Epstein-Barr virus (EBV), has frequently been implicated in the causation of reactive hemophagocytic syndrome (RHS) in the Western populations. EBV has also been implicated in the rare fulminant form of RHS occurring in Oriental children. However, our previous adult-predominant study indicated little clinical and serological evidence of EBV infection in patients with RHS in Hong Kong. In the present study, we further examined this issue using a more sensitive and specific technique for the demonstration of EBV, ie, in situ hybridization for EBV encoded RNA (EBER). The 43 Chinese patients studied were mostly adults with a mean age of 44 years, and a male to female ratio of 1.5:1. About two-thirds (28) of patients had associated malignant lymphoma at the time of diagnosis. Five patients had documented infection (typhoid fever 2; systemic candidiasis 1; adenovirus pneumonia 1; viral encephalitis 1), and two had systemic lupus erythematosus. EBER signals were detected in only 11 cases (25.6%). All positive cases were associated with malignant lymphoma, and the positive signals were exclusively localized to the lymphoma cells but not in the histiocytes. On comparing the results (11 of 28 cases positive; 39.3%) with our previous data on EBER-expression in malignant lymphomas in Hong Kong, no significant difference is observed in the frequency of EBV-positivity between the two groups of lymphomas. Thus, a definite pathogenetic link between EBV and lymphoma-associated RHS cannot be established. However, the overrepresentation of T and T/NK lineage lymphoma in this sample of lymphoma-associated with RHS (61%) versus nonselected cases of lymphomas (31%) suggests that it is the T and T/NK cell origin of the lymphoma rather than the EBV positivity that predisposes to RHS. Notwithstanding the previous findings, EBER in situ hybridization may still serve as a useful adjunct in the investigation of patients with RHS, because the presence of EBER-positive cells should raise a strong suspicion of an underlying malignant lymphoma.
疱疹病毒组，尤其是爱泼斯坦-巴尔病毒（EBV），在西方人群中常被认为与反应性噬血细胞综合征（RHS）的病因有关。EBV也与东方儿童中罕见的暴发性RHS有关。然而，我们之前以成人为主的研究表明，香港RHS患者几乎没有EBV感染的临床和血清学证据。在本研究中，我们使用一种更敏感、特异的技术来检测EBV，即EBV编码RNA（EBER）原位杂交，进一步研究了这个问题。所研究的43例中国患者大多为成年人，平均年龄44岁，男女比例为1.5:1。约三分之二（28例）患者在诊断时伴有恶性淋巴瘤。5例患者有明确的感染（伤寒热2例；系统性念珠菌病1例；腺病毒肺炎1例；病毒性脑炎1例），2例患有系统性红斑狼疮。仅在11例（25.6%）中检测到EBER信号。所有阳性病例均与恶性淋巴瘤有关，阳性信号仅局限于淋巴瘤细胞，而不在组织细胞中。将结果（28例中有11例阳性；39.3%）与我们之前关于香港恶性淋巴瘤中EBER表达的数据进行比较，两组淋巴瘤的EBV阳性频率无显著差异。因此，无法确立EBV与淋巴瘤相关RHS之间明确的致病联系。然而，在该RHS相关淋巴瘤样本中T和T/NK谱系淋巴瘤的比例过高（61%），而在非选择性淋巴瘤病例中为31%，这表明是淋巴瘤的T和T/NK细胞起源而非EBV阳性易导致RHS。尽管有先前的研究结果，EBER原位杂交在RHS患者的调查中仍可能是一种有用的辅助手段，因为EBER阳性细胞的存在应强烈怀疑存在潜在的恶性淋巴瘤。
