Garand R, Voisin S, Papin S, Praloran V, Lenormand B, Favre M, Philip P, Bernier M, Vanhaecke D, Falkenrodt A
Laboratoire d'Hématologie, Hôtel Dieu, Nantes, France.
Leukemia. 1993 Feb;7(2):161-7.
A group of 30 acute lymphoblastic leukemias (ALL) with the early pro-T phenotype CD7+/cCD3+/CD1-/CD3-/CD4-/CD8-were identified among 103 newly diagnosed ALL with T-lineage markers (T-ALL). Pro T-ALL was more often observed in adults, and showed a lower incidence of hyperleukocytosis than more mature T-ALL. Mediastinal masses and polar acid phosphatase positivity in blast cells were however observed with the same frequency in pro T-ALL and late T-ALL, and rearrangements of both T-cell receptor (TCR) beta and gamma genes were observed in half the pro T-ALL cases tested. The expression of CD34, DR, and myeloid (My) markers was significantly more frequent in pro T-ALL than in late T-ALL, and these three features were strongly linked. TCR gene rearrangements were two to three times more frequent in CD34- and My-pro T-ALL. However, both CD34+ and My+ pro T-ALL showed an incidence of mediastinal masses and polar acid phosphatase positivity similar to this observed in CD34- and My- cases. This supports the assumption that both types of ALL indeed are engaged in the T-lineage, and confirms intracytoplasmic cCD3 as the earliest marker for this lineage. Moreover, CD34 appears to persist up to an early stage of T-cell maturation, where the cells retain myeloid potentiality. Loss of CD34 correlates with TCR-beta gene rearrangement and definitive commitment to the T lineage. Event-free survival analysis suggested a poorer outcome for pro T-ALL in adult patients.
在103例新诊断的具有T系标志物（T-ALL）的急性淋巴细胞白血病中，鉴定出一组30例具有早期前T表型CD7+/cCD3+/CD1-/CD3-/CD4-/CD8-的急性淋巴细胞白血病。前T-ALL在成人中更常被观察到，与更成熟的T-ALL相比，白细胞增多症的发生率较低。然而，在前T-ALL和晚期T-ALL中，纵隔肿块和原始细胞中酸性磷酸酶阳性的发生率相同，并且在一半检测的前T-ALL病例中观察到T细胞受体（TCR）β和γ基因的重排。CD34、DR和髓系（My）标志物在前T-ALL中的表达明显比晚期T-ALL更频繁，并且这三个特征密切相关。TCR基因重排在CD34-和My-前T-ALL中更频繁，是其他类型的两到三倍。然而，CD34+和My+前T-ALL中纵隔肿块和酸性磷酸酶阳性的发生率与CD34-和My-病例中观察到的相似。这支持了两种类型的ALL确实都属于T系的假设，并证实细胞质内cCD3是该谱系的最早标志物。此外，CD34似乎一直持续到T细胞成熟的早期阶段，此时细胞保留髓系潜能。CD34的缺失与TCR-β基因重排以及对T系的最终确定有关。无事件生存分析表明，成人患者前T-ALL的预后较差。
