Dreazen O, Klisak I, Jones G, Ho W G, Sparkes R S, Gale R P
Department of Medicine, UCLA School of Medicine.
Br J Haematol. 1987 Nov;67(3):319-24. doi: 10.1111/j.1365-2141.1987.tb02353.x.
The Ph1 chromosome is present in 95% of patients with chronic myelogenous leukaemia (CML). The Ph1 chromosome also occurs in 5-25% of children and adults with acute lymphoblastic leukaemia (ALL). This observation raises questions as to whether these diseases are similar or identical. In patients with CML the c-abl and bcr genes are translocated and abnormally expressed. We studied molecular events related to bcr and c-abl in five patients with ALL to determine its relationship to CML. Four had the Ph1 chromosome; the fifth a probable Ph1 chromosome. c-abl and bcr abnormalities identical to CML were detected in four suggesting a common molecular basis. One patient with the Ph1 chromosome and c-abl translocation lacked these molecular changes but had abnormal c-abl gene transcription apparently unrelated to bcr. These data suggest that Ph1 chromosome positive ALL is heterogeneous; in some patients the molecular abnormality is identical to CML; in others c-abl is likewise involved but via a different mechanism.
95%的慢性粒细胞白血病(CML)患者存在Ph1染色体。5% - 25%的儿童和成人急性淋巴细胞白血病(ALL)患者也会出现Ph1染色体。这一观察结果引发了关于这些疾病是相似还是相同的疑问。在CML患者中,c-abl和bcr基因发生易位并异常表达。我们研究了5例ALL患者中与bcr和c-abl相关的分子事件,以确定其与CML的关系。4例有Ph1染色体;第5例可能有Ph1染色体。在4例中检测到与CML相同的c-abl和bcr异常,提示存在共同的分子基础。1例有Ph1染色体和c-abl易位的患者缺乏这些分子变化,但有明显与bcr无关的c-abl基因转录异常。这些数据表明,Ph1染色体阳性的ALL具有异质性;在一些患者中,分子异常与CML相同;在另一些患者中,c-abl同样受累,但机制不同。