Yatabe Y, Mori N, Oka K, Iijima T, Saga S, Takada K, Asai J
First Department of Pathology, Nagoya University, School of Medicine, Japan.
Arch Pathol Lab Med. 1995 May;119(5):409-17.
We report five childhood cases of fatal Epstein-Barr virus-associated lymphoproliferative disorder to determine their immunopathologic and immunogenotypic features.
Clinicopathologic features are described, using clinical, histologic, immunophenotypic, and genotypic examinations.
Autopsy cases were performed at Nagoya and Tsukuba university hospitals, Japan.
Infiltrating lymphocytes, which were positive for the EBV genome by in situ hybridization, were polymorphic and showed polyclonal immunoglobulin staining in all five cases. Two cases, however, showed genetic clonality of EBV termini. In one case, the clonality was observed only in the spleen, and in the other case, clonal rearrangement of the JH gene was also found. The former case showed none of the morphologic features of neoplasia and presented a morphology similar to virus-associated hemophagocytic syndrome. In the latter case, infiltrating lymphoid cells were much less polymorphous, and nodular masses of the lymphoid cells in colon and lung specimens were observed.
We suggest that some polyclonal EBV-associated lymphoproliferative disorders develop into polymorphic, but genotypically monoclonal, lymphoproliferative disorders.
我们报告5例儿童致命性EB病毒相关淋巴增殖性疾病病例，以确定其免疫病理和免疫基因型特征。
采用临床、组织学、免疫表型和基因分型检查描述临床病理特征。
尸检病例在日本名古屋大学医院和筑波大学医院进行。
通过原位杂交检测，浸润淋巴细胞的EB病毒基因组呈阳性，所有5例均表现为多形性，并显示多克隆免疫球蛋白染色。然而，2例显示EB病毒末端的基因克隆性。其中1例仅在脾脏中观察到克隆性，另一例还发现JH基因的克隆重排。前1例未显示肿瘤的形态学特征，呈现出与病毒相关噬血细胞综合征相似的形态。后1例中，浸润的淋巴细胞多形性明显减少，在结肠和肺标本中观察到淋巴细胞的结节状肿块。
我们认为一些多克隆EB病毒相关淋巴增殖性疾病会发展为多形性但基因单克隆的淋巴增殖性疾病。
