Prejzner Witold
Department of Hematology, Medical University of Gdańsk, Poland.
Med Sci Monit. 2002 May;8(5):BR193-7.
Chronic myeloid leukemia is characterized by the presence of the Philadelphia chromosome. At the molecular level a fusion of part of the ABL and BCR genes is observed. The breakpoint locations in the BCR gene fall between exons b2a2 or b3a2 (5' and 3', respectively). Depending on the BCR gene breakpoint two types of mRNA are created. Differences in the types of transcripts and/or the breakpoint site may have an influence on the clinical course of the disease. This prompted the present author to separate subtypes of chronic myeloid leukemia on the molecular level.
MATERIAL/METHODS: 71 patients diagnosed with chronic myeloid leukemia in the chronic phase were enrolled in the study. In 61 patients the type of BCR/ABL transcript was determined, and in 27 patients BCR breakpoints were established. Possible correlations between the clinical course, prognostic indexes, survival and the type of transcript and breakpoint were examined.
No correlation between the clinical course, prognostic index, or survival was observed in patients with 5' and 3' breakpoints. The patients with b3a2 transcript experienced longer survival than the patients expressing b2a2 transcript. However, no significant differences were observed in the duration of the chronic phase between the two groups.
The type of BCR gene breakpoint seems to have no prognostic value in patients with chronic myeloid leukemia. The longer survival of patients expressing the b3a2 transcript may be caused by the less aggressive course of the accelerated or blastic phase.
慢性髓性白血病的特征是存在费城染色体。在分子水平上,可观察到ABL和BCR基因部分的融合。BCR基因中的断点位置位于外显子b2a2或b3a2之间(分别为5'和3')。根据BCR基因断点可产生两种类型的mRNA。转录本类型和/或断点位置的差异可能会影响疾病的临床进程。这促使作者在分子水平上区分慢性髓性白血病的亚型。
材料/方法:71例诊断为慢性期慢性髓性白血病的患者纳入研究。确定了61例患者的BCR/ABL转录本类型,并确定了27例患者的BCR断点。研究了临床进程、预后指标、生存率与转录本类型和断点之间的可能相关性。
5'和3'断点患者的临床进程、预后指标或生存率之间未观察到相关性。b3a2转录本的患者比表达b2a2转录本的患者生存期更长。然而,两组慢性期的持续时间未观察到显著差异。
BCR基因断点类型似乎对慢性髓性白血病患者没有预后价值。表达b3a2转录本的患者生存期较长可能是由于加速期或急变期病程侵袭性较小。