Chubachi A, Imai H, Nishimura S, Saitoh M, Miura A B
Third Department of Internal Medicine, Akita University School of Medicine, Japan.
Arch Pathol Lab Med. 1992 Nov;116(11):1209-12.
A 53-year-old man with nasal T-cell lymphoma exhibited hemophagocytic syndrome as a terminal event. Immunohistochemical studies revealed that neoplastic cells were derived from T cells. Genotypic analysis of DNA samples that were obtained from the frozen tissue specimens demonstrated clonal rearrangements of the T-cell receptor beta-chain genes. No rearrangement was observed in the immunoglobulin heavy-chain gene. To our knowledge, only three cases of nasal T-cell lymphoma with hemophagocytic syndrome have been reported before the present case. All of these cases occurred in Oriental patients. The present report suggests the beneficial effect of high-dose glucocorticoid therapy on the prolongation of survival compared with that of the other three cases.
一名53岁的鼻型T细胞淋巴瘤男性患者在疾病终末期出现噬血细胞综合征。免疫组织化学研究显示肿瘤细胞来源于T细胞。对从冷冻组织标本中获取的DNA样本进行基因分型分析，结果显示T细胞受体β链基因存在克隆性重排。免疫球蛋白重链基因未观察到重排。据我们所知，在本病例之前仅有3例鼻型T细胞淋巴瘤合并噬血细胞综合征的病例报道。所有这些病例均发生在东方患者中。本报告提示，与其他3例病例相比，大剂量糖皮质激素治疗对延长生存期具有有益作用。
