Acute promyelocytic leukemia.
Acute promyelocytic leukemia.
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DOI：
10.1007/s11864-000-0013-1
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发表时间：
2000-04-01
影响因子：
4.3
通讯作者：
Douer, D
中科院分区：
文献类型：
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作者：
Douer, D
急性早幼粒细胞白血病（APL）的治疗不同于其他急性髓细胞白血病（AML）亚型。全反式维甲酸（ATRA）是所有新诊断APL患者标准缓解诱导的重要组成部分。同时给予ATRA和化疗的缓解诱导似乎与较少的复发有关。在不使用大剂量阿糖胞苷的情况下进一步巩固化疗，无病生存率可达70%至80%，其中许多患者治愈，比任何其他AML亚型都要多。APL对蒽环类药物特别敏感，化疗周期中应包括比其他AML亚型更高的剂量。低剂量化疗（每日口服6-巯基嘌呤，每周甲氨蝶呤）或全反式维甲酸维持治疗可进一步改善长期预后。新的方法也可用于复发患者，尽管最佳治疗方法尚不清楚。首次复发时未接受口服ATRA的患者可以使用这种药物治疗，首次复发的患者已经停药超过1年。由于缓解率低，ATRA不应用于第二次或随后复发的患者（无论过去是否给予ATRA），ATRA停药后早期复发的患者，或ATRA治疗期间复发的患者。也可以使用三氧化二砷，特别是在对ATRA耐药的患者中。由于三氧化二砷仍处于试验阶段，尚未广泛应用，因此不太可能对ATRA有反应或未成功接受ATRA再诱导的患者应接受化疗。ATRA或化疗诱导的第二次或后续缓解患者应接受巩固化疗。当三氧化二砷用于再诱导时，药物应持续几个周期;然而，增加巩固化疗可能会改善结果。由于目前尚不清楚APL在第二次或随后的缓解期是否可以通过挽救治疗治愈，异基因造血干细胞移植通常被考虑用于人类白细胞抗原（HL-A）相同的同胞和自体移植时，供体不存在的患者。然而，与新的治疗方法相比，移植对复发的作用尚不清楚。在第一次缓解时，移植没有作用。一种新的静脉注射ATRA的脂质体制剂正在研究中，似乎对晚期首次复发有效，它可能能够诱导和维持选定的患者在没有化疗的情况下首次缓解。
The treatment of acute promyelocytic leukemia (APL) is different from other subtypes of acute myelocytic leukemia (AML). All trans-retinoic acid (ATRA) is an essential component of the standard remission induction for all newly diagnosed APL patients. Remission induction with ATRA and chemotherapy given concurrently appears to be associated with fewer relapses. With further consolidation chemotherapy without high-dose cytosine arabinoside, the disease-free survival rate can reach 70% to 80%, and many of these patients are cured, more so than in any other AML subtype. APL is especially sensitive to anthracyclines, which should be included in the chemotherapy cycles at a higher dose than in other AML subtypes. Maintenance with low-dose chemotherapy (oral daily 6-mercaptopurine with weekly methotrexate) or ATRA further improves the long-term outcome. New approaches are also available for relapsing patients, although the optimal treatment is unknown. Patients who did not receive oral ATRA in first relapse can be treated with this agent, as can first relapsing patients who have been off the drug for more than 1 year. Because of poor remission rates, ATRA should not be used in patients with second or subsequent relapses (whether ATRA was given in the past), in patients with relapses early after ATRA discontinuation, or in patients relapsing while on ATRA therapy. Arsenic trioxide can also be used, especially in patients resistant to ATRA. Because arsenic trioxide is still experimental and not yet widely available, patients who are unlikely to respond to ATRA or who unsuccessfully undergo ATRA reinduction should be treated with chemotherapy. Patients in second or subsequent remission induced with ATRA or chemotherapy should receive consolidation chemotherapy. When arsenic trioxide is used for reinduction, the drug should be continued for several cycles; however, adding consolidation chemotherapy might improve the results. Because it is unknown whether APL in second or subsequent remission is curable with salvage therapy, allogeneic hematopoietic stem cell transplantation is often considered for patients with a human leukocyte antigen (HL-A)-identical sibling and autologous transplantation when a donor does not exist. However, compared with the new treatments, the role of transplantation for relapse is unclear. In first remission, there is no role for transplantation. A new liposomal formulation of intravenous ATRA is being investigated and seems effective in late first relapses, and it may be able to induce and maintain first remissions in selected patients without chemotherapy.