Heerema N A
Cytogenetics Laboratory, Indiana University School of Medicine, Indianapolis.
Hematol Oncol Clin North Am. 1990 Aug;4(4):795-820.
The chromosomal abnormalities that occur in acute lymphoblastic leukemia have greatly elucidated the biologic causes (leukemogenesis) of this disease. ALL is extremely heterogeneous; different cell types at differing stages of differentiation may become leukemic. Although not yet completely understood, the chromosomal abnormalities occurring in this disease are not random. Rather, the abnormalities are highly specific, with specific abnormalities occurring in specific cell phenotypes. Early chromosomal investigations were concerned with identification of heterogeneous cytogenetic "groups," with independent prognostic indications, among patients with ALL. These studies progressed to the identification of specific chromosomal translocations, many of which can be correlated with specific cell phenotypes. Molecular studies of these cytogenetic aberrations have defined specific chromosomal breakpoints, and specific genes, which have been implicated in the oncogenic process. As a result of these translocations, the involved genes either produce abnormal products or are deregulated. The products of these genes vary, but all are concerned with cell growth and differentiation. Much remains to be determined regarding cytogenetic abnormalities in acute lymphoblastic leukemia and their clinical and biologic significance. The molecular bases of all of the recurring abnormalities in ALL have not been determined. The biologic causes for the chromosomal abnormalities are not known, nor is it known why specific chromosomal aberrations correlate with prognosis.
急性淋巴细胞白血病中出现的染色体异常极大地阐明了这种疾病的生物学病因(白血病发生)。急性淋巴细胞白血病具有高度异质性;处于不同分化阶段的不同细胞类型都可能发生癌变。尽管尚未完全了解,但这种疾病中出现的染色体异常并非随机发生。相反,这些异常具有高度特异性,特定的异常出现在特定的细胞表型中。早期的染色体研究关注的是在急性淋巴细胞白血病患者中识别具有独立预后指标的异质性细胞遗传学“组”。这些研究进而发展到识别特定的染色体易位,其中许多易位可与特定的细胞表型相关联。对这些细胞遗传学畸变的分子研究确定了特定的染色体断点和特定的基因,这些基因与致癌过程有关。由于这些易位,相关基因要么产生异常产物,要么调控失常。这些基因的产物各不相同,但都与细胞生长和分化有关。关于急性淋巴细胞白血病中的细胞遗传学异常及其临床和生物学意义,仍有许多有待确定。急性淋巴细胞白血病中所有复发性异常的分子基础尚未确定。染色体异常的生物学原因尚不清楚,也不清楚为什么特定的染色体畸变与预后相关。