Ohyashiki K, Ohyashiki J H, Tauchi T, Fujieda H, Hojo H, Ohtaka M, Saito M, Nakazawa S, Toyama K
First Department of Internal Medicine, Tokyo Medical College, Japan.
Leukemia. 1991 Jul;5(7):611-4.
Acute lymphoblastic leukemia (ALL) patients with a Philadelphia chromosome (Ph+ ALL) were treated with a combination of antineoplastic drugs recommended for both myeloid and lymphoid leukemia (BHAC-DMPV: behenoylcytosine arabinoside, daunorubicin, 6-mercaptopurine, prednisolone, and vincristine). Ph+ ALL patients with chromosome breaks which occur within the major breakpoint cluster region (M-BCR rearranged Ph+ ALL) were treated with natural interferon-alpha (IFN-alpha) after entering complete remission. In this study, four of seven patients with Ph+ ALL had M-BCR rearrangement, and all achieved complete remission with karyotypic normalization. Subsequent cytogenetic analysis during complete remission in two ALL patients with M-BCR rearrangement revealed that the percentage of bone marrow cells with the Ph chromosome increased, while the bone marrow maintained remission status. This cytogenetic-hematological discrepancy led us to consider that M-BCR rearranged Ph+ ALL might be a variant of chronic myelogenous leukemia, therefore, three Ph+ ALL patients with M-BCR rearrangement were treated with IFN-alpha after achieving complete remission. In contrast, only one of three patients with M-BCR non-rearranged Ph+ ALL obtained complete remission.
患有费城染色体的急性淋巴细胞白血病（Ph+ ALL）患者接受了推荐用于髓系和淋巴系白血病的抗肿瘤药物联合治疗（BHAC-DMPV：山嵛酰阿糖胞苷、柔红霉素、6-巯基嘌呤、泼尼松龙和长春新碱）。在主要断裂点簇区域内发生染色体断裂的Ph+ ALL患者（M-BCR重排的Ph+ ALL）在进入完全缓解后接受天然α干扰素（IFN-α）治疗。在本研究中，7例Ph+ ALL患者中有4例发生M-BCR重排，且所有患者均实现了核型正常化的完全缓解。随后，对2例M-BCR重排的ALL患者完全缓解期间进行的细胞遗传学分析显示，携带费城染色体的骨髓细胞百分比增加，而骨髓维持缓解状态。这种细胞遗传学-血液学差异使我们认为M-BCR重排的Ph+ ALL可能是慢性粒细胞白血病的一种变体，因此，3例M-BCR重排的Ph+ ALL患者在实现完全缓解后接受了IFN-α治疗。相比之下，3例M-BCR未重排的Ph+ ALL患者中只有1例获得完全缓解。
