III Medizinische Klinik, Medizinische Fakultät Mannheim, Ruprecht-Karls-Universität, Heidelberg, Germany.
Blood. 2012 Jul 26;120(4):737-47. doi: 10.1182/blood-2012-03-380147. Epub 2012 May 31.
Blast crisis (BC) remains the major challenge in the management of chronic myeloid leukemia (CML). It is now generally accepted that BC is the consequence of continued BCR-ABL activity leading to genetic instability, DNA damage, and impaired DNA repair. Most patients with BC carry multiple mutations, and up to 80% show additional chromosomal aberrations in a nonrandom pattern. Treatment with tyrosine kinase inhibitors has improved survival in BC modestly, but most long-term survivors are those who have been transplanted. Patients in BC should be treated with a tyrosine kinase inhibitor according to mutation profile, with or without chemotherapy, with the goal of achieving a second chronic phase and proceeding to allogeneic stem cell transplantation as quickly as possible. Although long-term remissions are rare, allogeneic stem cell transplantation provides the best chance of a cure in BC. Investigational agents are not likely to provide an alternative in the near future. In view of these limited options, prevention of BC by a rigorous and early elimination of BCR-ABL is recommended. Early response indicators should be used to select patients for alternative therapies and early transplantation. Every attempt should be made to reduce or eliminate BCR-ABL consistent with good patient care as far as possible.
急变期（BC）仍然是慢性髓性白血病（CML）治疗的主要挑战。现在普遍认为，BC 是由于持续的 BCR-ABL 活性导致遗传不稳定性、DNA 损伤和受损的 DNA 修复。大多数 BC 患者携带多种突变，高达 80%的患者表现出非随机模式的额外染色体异常。酪氨酸激酶抑制剂的治疗适度改善了 BC 的生存率，但大多数长期幸存者是那些接受过移植的患者。根据突变谱，BC 患者应使用酪氨酸激酶抑制剂进行治疗，联合或不联合化疗，目标是实现第二次慢性期，并尽快进行异基因干细胞移植。尽管长期缓解很少见，但异基因干细胞移植为 BC 提供了治愈的最佳机会。在不久的将来，研究药物不太可能提供替代方案。鉴于这些有限的选择，建议通过严格和早期消除 BCR-ABL 来预防 BC。应使用早期反应指标来选择接受替代治疗和早期移植的患者。应尽一切努力尽可能减少或消除 BCR-ABL，同时为患者提供良好的护理。
