Pollicardo N, O'Brien S, Estey E H, al-Bitar M, Pierce S, Keating M, Kantarjian H M
Department of Hematology, MD Anderson Cancer Center, Houston, Texas 77030, USA.
Leukemia. 1996 Jan;10(1):27-31.
The aims of this study were to analyze the characteristics and outcome of patients with secondary acute promyelocytic leukemia (APL) and compare them to those with primary APL. One hundred and thirteen patients referred to our service with a diagnosis of APL were reviewed. Fourteen were classified as secondary APL. Nine were induced with chemotherapy, and five with all-trans retinoic acid plus chemotherapy. Pretreatment characteristics, response to therapy and outcome of primary vs secondary APL were compared by standard statistical methods. Secondary APL constituted 12% of all APL cases. Patients with secondary APL were significantly older (median age 56 vs 36 years; P < 0.01) and had a lower incidence of hypofibrinogenemia (P < 0.01) than those with primary APL. The complete response (CR) rates were similar with secondary vs primary APL (CR rates 79 vs 69%), as were CR duration and survival. The CR rates at 5 years were 57 and 45%, respectively(P not significant), and the survival rates 37 and 35%, respectively (P not significant). The incidence of secondary APL within APL disease (12%) was similar to the incidence of secondary acute myeloid leukemia (AML) in karyotypes not known to be therapy-related (diploid, t(8;21), inversion 16: incidences 9 to 12%), but was significantly lower than in karyotypes known to be therapy-related (chromosome 5 or 7 abnormalities, 11q; incidences 30 and 33%). We conclude that secondary APL has general characteristics and outcome similar to primary APL. it is more likely a second primary rather than therapy-related AML, and should be treated in a manner similar to primary APL.
本研究旨在分析继发性急性早幼粒细胞白血病（APL）患者的特征及预后，并与原发性APL患者进行比较。对我院收治的113例诊断为APL的患者进行了回顾性分析。其中14例被归类为继发性APL。9例接受化疗诱导，5例接受全反式维甲酸联合化疗。采用标准统计学方法比较原发性与继发性APL的预处理特征、治疗反应及预后。继发性APL占所有APL病例的12%。继发性APL患者比原发性APL患者年龄显著更大（中位年龄56岁对36岁；P<0.01），低纤维蛋白原血症发生率更低（P<0.01）。继发性与原发性APL的完全缓解（CR）率相似（CR率分别为79%和%69），CR持续时间及生存率也相似。5年时的CR率分别为57%和45%（P无统计学意义），生存率分别为37%和35%（P无统计学意义）。APL疾病中继发性APL的发生率（12%）与核型未知为治疗相关的继发性急性髓系白血病（AML）（二倍体、t(8;21)、16号染色体倒位：发生率9%至12%）相似，但显著低于已知为治疗相关的核型（5号或7号染色体异常、11q；发生率30%和33%）。我们得出结论，继发性APL具有与原发性APL相似的一般特征和预后。它更可能是第二种原发性疾病而非治疗相关的AML，应以类似于原发性APL的方式进行治疗。
