Kojima K, Kobayashi H, Imoto S, Nakagawa T, Matsui T, Kawachi Y, Oda K, Yano T, Kobayashi H, Noguchi M, Hara M, Oshimi K
Division of Hematology, Ehime Prefectural Central Hospital, Japan.
Int J Hematol. 1998 Oct;68(3):291-6. doi: 10.1016/s0925-5710(98)00074-7.
We studied ten cases of Japanese T-cell prolymphocytic leukemia (T-PLL) collected over the last 9 years. Median age was 61 years with a male predominance (M:F, 8:2). The main disease features were splenomegaly, lymphadenopathy, hepatomegaly, skin lesions and serous effusions. The clinical course was progressive with a median survival of 10 months. Immunophenotyping showed that the prolymphocytes had a post-thymic phenotype (TdT-, CD1a-, CD2+, CD3+, CD5+, CD7+) with a predominant CD4+ immunophenotype. Cytogenetic analysis showed no consistent abnormalities. 14q abnormality and trisomy 8q, which are frequently seen in T-PLL of Western countries, were found in only two and zero cases, respectively. We conclude that the clinical and biological characteristics of T-PLL in Japan are almost the same as those in Western countries. However, the cytogenetic findings of T-PLL in Japan might be different.
我们研究了过去9年中收集的10例日本T细胞幼淋巴细胞白血病(T-PLL)病例。中位年龄为61岁,男性占优势(男:女,8:2)。主要疾病特征为脾肿大、淋巴结病、肝肿大、皮肤病变和浆膜腔积液。临床病程呈进行性,中位生存期为10个月。免疫表型分析显示幼淋巴细胞具有胸腺后表型(末端脱氧核苷酸转移酶阴性、CD1a阴性、CD2阳性、CD3阳性、CD5阳性、CD7阳性),以CD4+免疫表型为主。细胞遗传学分析未发现一致的异常情况。在西方国家T-PLL中常见的14q异常和8q三体,在本研究中分别仅在2例和0例中发现。我们得出结论,日本T-PLL的临床和生物学特征与西方国家几乎相同。然而,日本T-PLL的细胞遗传学结果可能有所不同。