O'Brien S, Witt R, Talpaz M, Kantarjian H
Department of Hematology, University of Texas M. D. Anderson Cancer Center, Houston.
Cancer. 1991 Apr 1;67(7):1946-9. doi: 10.1002/1097-0142(19910401)67:7<1946::aid-cncr2820670720>3.0.co;2-#.
Treatment of Philadelphia (Ph) chromosome-positive chronic myelogenous leukemia (CML) with recombinant interferon-alpha (IFN-A) results in complete disappearance of the Ph chromosome in about 10% to 15% of patients in early chronic phase. This group has a long survival and very low incidence of blast crisis. The first known case is reported of extramedullary blastic transformation in a patient with medullary complete cytogenetic response (0% Ph-positive metaphases) to IFN-A. Four episodes of extramedullary blast crisis have occurred in this patient. The first three episodes were lymphoid by morphology and cytochemical stains. Molecular analysis confirmed breakpoint cluster region rearrangement. The most recent transformation was myeloid in nature and involved bone and pulmonary parenchyma. The patient is currently undergoing a second autologous transplantation with stored bone marrow that is Ph negative. The patient has survived more than 18 months since the first episode of blast crisis, and the bone marrow is normal.
用重组α干扰素（IFN-A）治疗费城（Ph）染色体阳性的慢性粒细胞白血病（CML），在慢性期早期约10%至15%的患者中可使Ph染色体完全消失。该组患者生存期长，急变期发生率极低。本文报告了首例经IFN-A治疗达到骨髓完全细胞遗传学缓解（0% Ph阳性中期分裂相）的患者发生髓外原始细胞转化的病例。该患者发生了4次髓外急变期。前三期经形态学和细胞化学染色显示为淋巴细胞性。分子分析证实有断裂点簇集区重排。最近一次转化为髓细胞性，累及骨骼和肺实质。该患者目前正在接受第二次自体骨髓移植，所储存的骨髓为Ph阴性。自首次急变期发作以来，该患者已存活超过18个月，骨髓正常。
