Karbasian Esfahani Merat, Morris Evelyn L, Dutcher Janice P, Wiernik Peter H
Our Lady of Mercy Comprehensive Cancer Center, 600 East 233rd Street, Bronx, NY 10466, USA.
Curr Treat Options Oncol. 2006 May;7(3):189-99. doi: 10.1007/s11864-006-0012-y.
Chronic myelogenous leukemia (CML), also known as chronic myelocytic or chronic myeloid leukemia, is a clonal disorder of hematopoiesis that arises in a hematopoietic stem cell or early progenitor cell. This is characterized by the dysregulated production of mature nonlymphoid cells with normal differentiation. Eventually, in spite of the term chronic, there is progression to acute leukemia, usually of the myeloid variety, which is highly resistant to current therapies. Despite recent improvements in the treatment of early-stage disease, CML blast crisis (CMLBC) remains a therapeutic challenge. CMLBC is highly refractory to standard induction chemotherapy, with a response rate in myeloid blast crisis of less than 30%. Conventional chemotherapy has been much less successful in this disease compared with de novo acute leukemia, with a mean survival after diagnosis of blast crisis of only 2 to 4 months for nonresponders. Many regimens of chemotherapies have been tried in CMLBC, with minor success. Although imatinib was evaluated in patients with CMLBC, most CMLBC cases today arise in patients already on imatinib-based therapy and developing blastic phase on that therapy; thus there is no standard therapy for patients with CMLBC. Further studies of the mechanisms of transformation of chronic-phase CMLBC at a molecular level, and methods to target these molecular abnormalities, will determine the future direction of new treatment modalities. The prognosis of CML in blast crisis remains disappointing, despite great efforts. Currently, the most successful strategy for improving survival in CML is by prolonging the chronic phase and delaying the onset of blast crisis.
慢性粒细胞白血病（CML），也称为慢性髓细胞性或慢性髓样白血病，是一种造血克隆性疾病，起源于造血干细胞或早期祖细胞。其特征是成熟非淋巴细胞的产生失调，但分化正常。最终，尽管有“慢性”一词，但仍会进展为急性白血病，通常是髓细胞性的，对当前疗法具有高度抗性。尽管近期早期疾病的治疗有所改善，但慢性粒细胞白血病急变期（CMLBC）仍然是一个治疗挑战。CMLBC对标准诱导化疗高度难治，髓细胞急变期的缓解率低于30%。与原发性急性白血病相比，传统化疗在这种疾病中的效果要差得多，对于无反应者，急变期诊断后的平均生存期仅为2至4个月。在CMLBC中尝试了许多化疗方案，但收效甚微。尽管对伊马替尼在CMLBC患者中进行了评估，但如今大多数CMLBC病例发生在已经接受基于伊马替尼治疗且在此治疗过程中进入急变期的患者中；因此，对于CMLBC患者没有标准疗法。在分子水平上进一步研究慢性期CMLBC转化的机制以及针对这些分子异常的方法，将决定新治疗模式的未来方向。尽管付出了巨大努力，但CML急变期的预后仍然令人失望。目前，改善CML患者生存的最成功策略是延长慢性期并延迟急变期的 onset 。（原文中onset似乎有误，推测可能是onset of blast crisis，直译为“急变期的发作”，这里按推测意译为“急变期的出现”） 急变期的出现。
