Yamamoto T, Shirakawa A, Kawaguchi M, Masuda A, Nishikawa T, Kobayashi M
Department of Pathology and Surgical Pathology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, 162-8666 Tokyo, Japan.
Ann Hematol. 2004 Feb;83(2):127-32. doi: 10.1007/s00277-003-0776-0. Epub 2003 Oct 3.
This report describes lytic infection with Epstein-Barr virus (EBV) in three cases of hemophagocytic syndrome (HPS) presumably associated with EBV-induced lymphoproliferative disorder. All cases were previously healthy females with ages ranging from 52 to 87 years who showed a fulminant clinical course with accompanying fever, liver dysfunction, and disseminated intravascular coagulation. Cases 1 and 2 showed proliferation of atypical T lymphocytes, and case 3 showed proliferation of atypical B lymphocytes. Hemophagocytic histiocytes were observed in the bone marrow, lymph nodes, spleen, and liver. Atypical lymphocytes in all cases showed a positive reaction for both EBV-encoded small RNA (EBER) indicating latent infection with EBV and immediate early mRNAs of the Bam HI fragment of lower stranded frame (BHLF), indicating lytic infection by in situ hybridization. Interestingly, BHLF-positive cells were predominant in all cases. It is possible that reactivation of EBV infection may be involved in triggering the induction of cytokines and abnormal activation of histiocytes.
本报告描述了3例噬血细胞综合征（HPS）中与爱泼斯坦-巴尔病毒（EBV）诱导的淋巴增殖性疾病相关的EBV溶解性感染。所有病例均为既往健康的女性，年龄在52至87岁之间，临床病程凶险，伴有发热、肝功能障碍和弥散性血管内凝血。病例1和病例2表现为非典型T淋巴细胞增殖，病例3表现为非典型B淋巴细胞增殖。在骨髓、淋巴结、脾脏和肝脏中均观察到噬血细胞组织细胞。所有病例中的非典型淋巴细胞通过原位杂交对EBV编码的小RNA（EBER）呈阳性反应，表明EBV潜伏感染，对较低链框Bam HI片段的立即早期mRNA（BHLF）呈阳性反应，表明溶解性感染。有趣的是，BHLF阳性细胞在所有病例中均占主导。EBV感染的重新激活可能参与触发细胞因子的诱导和组织细胞的异常激活。
