Oshimi Kazuo, Kawa Keisei, Nakamura Shigeo, Suzuki Ritsuro, Suzumiya Junji, Yamaguchi Motoko, Kameoka Junichi, Tagawa Shinichi, Imamura Nobutaka, Ohshima Koichi, Kojya Shizuo, Iwatsuki Keiji, Tokura Yoshiki, Sato Eriko, Sugimori Hiroki
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.
Hematology. 2005 Jun;10(3):237-45. doi: 10.1080/10245330400026162.
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 hemoglobin 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.
推定起源于前体和成熟自然杀伤（NK）细胞的肿瘤较为罕见，其临床特征尚不清楚。日本进行了一项全国性调查，以阐明1994年至1998年间诊断出的这些肿瘤的临床特征，并对237例符合推定NK细胞系肿瘤标准的患者的数据进行了分析。其中，11例为髓系/NK细胞前体急性白血病，15例为母细胞性NK细胞淋巴瘤，21例为前体NK细胞急性淋巴细胞白血病，22例为侵袭性NK细胞白血病/淋巴瘤，149例为鼻型NK细胞淋巴瘤（123例鼻腔型和26例鼻外型），19例为慢性NK淋巴细胞增多症。侵袭性NK细胞白血病/淋巴瘤患者的中位总生存时间为2个月，慢性NK淋巴细胞增多症患者的中位总生存时间超过8年，其他类型NK细胞肿瘤患者的中位总生存时间在6至22个月之间。鼻腔NK细胞淋巴瘤和鼻外型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，男性主要为鼻腔NK细胞淋巴瘤，女性主要为鼻外型NK细胞淋巴瘤。鼻外型NK细胞淋巴瘤患者往往表现出疾病进展更为严重的状态，国际预后指数和乳酸脱氢酶水平显著更高，血红蛋白和血小板水平显著更低。然而，总生存率没有显著差异。前体NK细胞急性淋巴细胞白血病和母细胞性NK细胞淋巴瘤是根据骨髓或外周血中母细胞是否占30%或更多来任意定义的，但在发病年龄、性别、受累部位或预后方面没有显著差异。侵袭性NK细胞白血病/淋巴瘤和鼻外型NK细胞淋巴瘤是根据骨髓或外周血中是否存在30%或更多的大颗粒淋巴细胞来任意定义的，侵袭性NK细胞白血病/淋巴瘤可能是鼻外型NK细胞淋巴瘤的白血病期。然而，鼻外型NK细胞淋巴瘤的皮肤受累发生率显著更高，表明这两种疾病是不同的。在鼻腔NK细胞淋巴瘤中，所有检测患者的肿瘤细胞中均检测到爱泼斯坦-巴尔病毒，提示其致病作用。
