NK-cell neoplasms in Japan
NK-cell neoplasms in Japan
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DOI：
10.1080/10245330400026162
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发表时间：
2005-06-01
期刊：
影响因子：
1.9
通讯作者：
Sugimori, H
中科院分区：
文献类型：
--
作者：
Oshimi, K;Kawa, K;Sugimori, H
肿瘤可能起源于前体和成熟的自然杀伤(NK)细胞是罕见的，其临床特征尚不清楚。为了阐明1994年至1998年间诊断的这些肿瘤的临床特征，在日本进行了一项全国性的调查，并分析了237名符合假定的NK细胞系肿瘤标准的患者的数据。其中髓系/NK细胞前体急性白血病11例，原始NK细胞淋巴瘤15例，NK细胞前体急性淋巴细胞白血病21例，侵袭性NK细胞白血病/淋巴瘤22例，鼻型NK细胞淋巴瘤149例(鼻型123例，鼻外26例)，慢性NK细胞增多症19例。侵袭性NK细胞白血病/淋巴瘤患者的中位生存期为2个月，慢性NK细胞增多症患者的中位生存期为8年以上，其他类型的NK细胞肿瘤患者的中位生存期为6~22个月。鼻NK细胞淋巴瘤和鼻外NK细胞淋巴瘤具有相同的组织学特征。发病年龄相同，但性别不同，男性以鼻NK细胞淋巴瘤为主，女性以鼻外NK细胞淋巴瘤为主。鼻外NK细胞淋巴瘤患者的病情越严重，国际预后指数和LDH水平越高，而血球蛋白和血小板水平越低。然而，总体存活率并没有显著差异。前体NK细胞急性淋巴细胞性白血病和原始NK细胞淋巴瘤以骨髓或外周血中有无30%或以上的原始细胞为标准，但受累年龄、性别、受累部位和预后无显著差异。侵袭性NK细胞白血病/淋巴瘤和鼻外NK细胞淋巴瘤的定义是骨髓或外周血中有或不存在30%或以上的大颗粒淋巴细胞，侵袭性NK细胞白血病/淋巴瘤可能是鼻外NK细胞淋巴瘤的白血病阶段。然而，鼻外NK细胞淋巴瘤皮肤受累的发生率显著高于鼻外NK细胞淋巴瘤，这表明这两种疾病是不同的。在鼻腔NK细胞淋巴瘤中，所有受试者的肿瘤细胞中均检测到EB病毒，提示其致病作用。
Neoplasms putatively originating from precursor and mature natural killer (NK) cells are rare, and their clinical features are unclear. A nationwide survey was performed in Japan to clarify the clinical features of these neoplasms diagnosed between 1994 and 1998, and data for 237 patients who met the criteria for putative NK cell-lineage neoplasms were analyzed. Among them, 11 had myeloid/NK-cell precursor acute leukemia, 15 blastic NK-cell lymphoma, 21 precursor NK-cell acute lymphoblastic leukemia, 22 aggressive NK-cell leukemia/lymphoma, 149 nasal-type NK-cell lymphoma (123 nasal and 26 extranasal) and 19 chronic NK lymphocytosis. The median overall survival time of patients with aggressive NK-cell leukemia/lymphoma was 2 months, which for chronic NK lymphocytosis was more than 8 years, and that for the other types of NK-cell neoplasms was between 6 and 22 months. Nasal NK-cell lymphoma and extranasal NK-cell lymphoma share the same histology. The age of affliction was the same, but the sex was different with males predominantly having nasal NK-cell lymphoma and females extranasal NK-cell lymphoma. Patients with extranasal NK-cell lymphoma had the tendency to exhibit a more advanced state of disease, with significantly higher International Prognostic Index and LDH levels, and significantly lower hemogolobin and platelet levels. The overall survival, however, did not differ significantly. Precursor NK-cell acute lymphoblastic leukemia and blastic NK-cell lymphoma were arbitrarily defined by the presence or absence of 30% or more of blastic cells in the bone marrow or peripheral blood, but there were no significant differences for affected age, gender, involved sites or prognosis. Aggressive NK-cell leukemia/lymphoma and extranasal NK-cell lymphoma were arbitrarily defined by the presence or absence of 30% or more of large granular lymphocytes in the bone marrow or peripheral blood and it is possible that aggressive NK-cell leukemia/lymphoma is a leukemic phase of extranasal NK-cell lymphoma. The incidence of skin involvement, however, was significantly higher for extranasal NK-cell lymphoma, suggesting that the two diseases are different. In nasal NK-cell lymphoma, Epstein-Barr virus in tumor cells was detected in all patients tested, suggesting its causative role.