Goldman J M
Royal Postgraduate Medical School, London.
Bone Marrow Transplant. 1989 Jan;4 Suppl 1:129-30.
The great majority of patients with chronic myeloid leukaemia (CML) have a Philadelphia (Ph) chromosome which has proved at molecular level to be associated with the production of chimeric BCR-ABL gene which in turn is expressed as a fusion protein (P210) with tyrosine kinase activity. An equivalent but somewhat smaller chimeric gene and fusion protein (P190) is found in some cases of Ph-positive acute leukaemia. Though the consistency of these abnormal findings in patients with Ph-positive leukaemia is strong evidence for their pathogenetic role, there are still many unanswered questions.
绝大多数慢性髓性白血病（CML）患者都有费城（Ph）染色体，在分子水平上已证实该染色体与嵌合BCR-ABL基因的产生有关，而该基因又表达为具有酪氨酸激酶活性的融合蛋白（P210）。在某些Ph阳性急性白血病病例中发现了一种等效但稍小的嵌合基因和融合蛋白（P190）。尽管这些异常发现在Ph阳性白血病患者中的一致性有力地证明了它们的致病作用，但仍有许多问题未得到解答。
