Roper M, Crist W M, Metzgar R, Ragab A H, Smith S, Starling K, Pullen J, Leventhal B, Bartolucci A A, Cooper M D
Blood. 1983 May;61(5):830-7.
Although childhood T-cell acute lymphocytic leukemia (T-ALL) and T-cell non-Hodgkin's lymphoma (T-NHL) have certain clinical features in common, T-ALL carries a notably poorer prognosis than does T-NHL. To determine whether the malignant cells from patients with these disorders are distinguishable, we examined bone marrow and/or blood from 51 children with T-ALL and tumor biopsy specimens from 17 with T-NHL, using a panel of monoclonal antibodies directed against T-cell differentiation antigens. We found considerable phenotypic heterogeneity in both T-ALL and T-NHL. Of the T-ALL (defined by greater than 25% blasts in the bone marrow) patients, 33% demonstrated a surface antigen pattern consistent with the earliest thymocyte stage of T-cell development (T9+ and/or T10+, or T6-/T4-/T8-/T3-), 37% were of a midthymocyte stage (T6+, and/or simultaneous expression of T4-helper and T8-suppressor antigens), and 30% expressed surface antigen patterns found on mature thymocytes (T3+, variable expression of other antigens). In contrast, tumor cell phenotypes in the 17 T-NHL patients were approximately equally distributed between mid- and mature thymocyte phenotypes. No NHL samples were classified as the early thymocyte phenotype. Clinical features as related to specific T-ALL immunophenotypes are presented, and the implications of these findings in regard to the current understanding of the differences in tumor biology between T-ALL and T-NHL are discussed.
尽管儿童T细胞急性淋巴细胞白血病(T-ALL)和T细胞非霍奇金淋巴瘤(T-NHL)有某些共同的临床特征,但T-ALL的预后明显比T-NHL差。为了确定这些疾病患者的恶性细胞是否可区分,我们使用一组针对T细胞分化抗原的单克隆抗体,检查了51例T-ALL患儿的骨髓和/或血液以及17例T-NHL患儿的肿瘤活检标本。我们发现T-ALL和T-NHL中均存在相当大的表型异质性。在T-ALL(定义为骨髓中原始细胞大于25%)患者中,33%表现出与T细胞发育最早胸腺细胞阶段一致的表面抗原模式(T9+和/或T10+,或T6-/T4-/T8-/T3-),37%处于胸腺细胞中期阶段(T6+,和/或同时表达T4辅助抗原和T8抑制抗原),30%表达成熟胸腺细胞上发现的表面抗原模式(T3+,其他抗原表达可变)。相比之下,17例T-NHL患者的肿瘤细胞表型在胸腺细胞中期和成熟表型之间大致均匀分布。没有NHL样本被归类为早期胸腺细胞表型。本文介绍了与特定T-ALL免疫表型相关的临床特征,并讨论了这些发现对当前理解T-ALL和T-NHL之间肿瘤生物学差异的意义。