Lin Chung-Wu, Yang Jia-Ying, Chuang Yi-Chun, Chen Yu-Hua, Albitar Maher, Hsu Su-Ming
Department of Pathology, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
Lab Invest. 2003 Jan;83(1):55-64. doi: 10.1097/01.lab.0000047491.62596.a3.
Most sinonasal lymphomas have a restricted killer immunoglobulin-like receptor (KIR) repertoire without a monoclonal T-cell receptor-gamma (TCR-gamma) rearrangement, implying an NK lineage. However, the lineage assignment of sinonasal lymphoma with a monoclonal TCR-gamma rearrangement is unclear because of its mixed NK/T phenotype. The possibility of a mixed NK/T lineage arises with the discovery of T cells with NK features, such as KIR(+) T cells or Valpha24(+) NKT cells. The former might transform into a T-cell lymphoma with both a monoclonal TCR-gamma rearrangement and a restricted KIR repertoire; the latter might give rise to a T-cell lymphoma with a monoclonal Valpha24 rearrangement and possibly a restricted KIR repertoire. To identify such mixed-lineage lymphomas, we undertook a survey of 15 consecutive sinonasal lymphomas and found six with both a restricted KIR repertoire and a monoclonal TCR-gamma rearrangement, consistent with KIR(+) T-cell lymphomas. Among these six cases, four female CD56(-)/CD44(-)/CD8(-)/CD45RO(+)/CD45RA(-) cases constituted a distinct group with a better prognosis than the rest of the male cases of sinonasal lymphomas. None of the six cases had a monoclonal Valpha24 repertoire, thus excluding a derivation from NKT cells. The predominance of KIR(+) T cells that normally function in chronic viral infections over Valpha24(+) NKT cells that typically recognize glycolipid antigens is consistent with the known association of Epstein-Barr virus infection with sinonasal lymphoma. The demonstration of mixed lineage in a mature lymphoid neoplasm is unusual and echoes the World Health Organization classification that placed NK-cell and T-cell lymphomas in a mixed group.
大多数鼻窦淋巴瘤具有受限的杀伤细胞免疫球蛋白样受体（KIR）谱，且无单克隆T细胞受体γ（TCR-γ）重排，提示为自然杀伤（NK）细胞谱系。然而，具有单克隆TCR-γ重排的鼻窦淋巴瘤因其混合性NK/T表型，其谱系归属尚不清楚。随着具有NK特征的T细胞的发现，如KIR(+) T细胞或Vα24(+) NKT细胞，出现了混合性NK/T谱系的可能性。前者可能转变为具有单克隆TCR-γ重排和受限KIR谱的T细胞淋巴瘤；后者可能产生具有单克隆Vα24重排且可能具有受限KIR谱的T细胞淋巴瘤。为了识别此类混合谱系淋巴瘤，我们对15例连续的鼻窦淋巴瘤进行了调查，发现6例具有受限的KIR谱和单克隆TCR-γ重排，符合KIR(+) T细胞淋巴瘤。在这6例病例中，4例女性CD56(-)/CD44(-)/CD8(-)/CD45RO(+)/CD45RA(-)病例构成一个独特的组，其预后优于其余男性鼻窦淋巴瘤病例。这6例病例均无单克隆Vα24谱，因此排除了源自NKT细胞的可能性。在慢性病毒感染中正常发挥作用的KIR(+) T细胞比通常识别糖脂抗原的Vα24(+) NKT细胞占优势，这与已知的爱泼斯坦-巴尔病毒感染与鼻窦淋巴瘤的关联一致。在成熟淋巴样肿瘤中证明混合谱系是不寻常的，这与世界卫生组织将NK细胞淋巴瘤和T细胞淋巴瘤归为混合组的分类相呼应。
