Acute promyelocytic leukemia: an experience on 95 greek patients treated in the all-trans-retinoic Acid era.
Acute promyelocytic leukemia: an experience on 95 greek patients treated in the all-trans-retinoic Acid era.
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DOI：
10.4084/mjhid.2011.053
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发表时间：
2011
影响因子：
3.2
通讯作者：
Harhalakis N
中科院分区：
文献类型：
--
作者：
Pagoni M;Garofalaki M;Panitsas F;Manola K;Psarra K;Economopoulos P;Vourtsi A;Antoniades M;Gkirkas K;Tzouvara E;Katis F;Prokopiou C;Tziotziou I;Balta A;Lemissiou E;Tsirigotis P;Repoussis P;Harhalakis N
急性早幼粒细胞白血病(APL)在全反式维甲酸(ATRA)和以蒽环类药物为基础的联合化疗中有很高的治愈率，但早期死亡率仍然很高。在本研究中，我们报告了在希腊和塞浦路斯的不同医院诊断和治疗的APL患者的临床、免疫表型、细胞遗传学和分子特征和结果。我们描述了在过去15年中诊断的95例APL患者的数据。7例(7.4%)新诊断的APL患者在发病72小时内死于颅内出血。除2例患者外，其余患者均用全反式维甲酸或全反式维甲酸加化疗。早期死亡率为14.9%。诱导后80例可评价患者血液学完全缓解。累积复发率为18.3%。10名复发患者中有8名成功抢救，而两名患有分子耐药性疾病的患者在抢救治疗中死亡。5年总生存率为78.4%，无瘤生存率为73.6%。在年龄超过60岁的OS的多因素分析中，确诊时的DIC和发病时轻微的大出血被确定为不良预后因素。在有Flt3突变状态数据的患者亚组中(94例中有49例)，在最终的OS模型中，ITD阳性也仍然是一个独立的预后因素，与大出血和略微高的Sanz评分一起。我们发现CD2的表达与辨证分型(DS)的发生发展密切相关。总而言之，APL患者治疗的主要问题仍然是高早期死亡率。
Acute promyelocytic leukemia (APL) is highly curable with the combination of all-transretinoic acid (ATRA) and anthracycline based chemotherapy, but the percentage of early deaths remains high. In the present study, we report the clinical, immunophenotypic, cytogenetic and molecular characteristics and outcome of APL patients diagnosed and treated in various Hospitals of Greece and Cyprus. We describe the data of ninety-five APL patients who were diagnosed during the last 15 years. Seven (7.4%) newly diagnosed APL patients died due to intracranial hemorrhage within 72 hours of presentation. All but two patients were induced with ATRA alone or ATRA plus chemotherapy. The early death rate was 14.9%. After induction all 80 evaluable patients achieved complete hematologic remission. The cumulative incidence of relapse was 18.3%. Eight of the ten relapsed patients were successfully salvaged, while both patients with molecularly resistant disease died during salvage treatment. Overall survival (OS) at 5 years was 78.4% and disease free survival (DFS) 73.6%. In multivariate analysis of OS age over 60 years, DIC at diagnosis and marginally major hemorrhage at presentation were identified as adverse prognostic factors. In the subgroup of patients with available data on FLT3 mutation status (49 out of 94), ITD positivity also remained as an independent prognostic factor in the final model of OS, together with major hemorrhage and marginally high Sanz score. We found a close correlation between the CD2 expression and the development of the differentiation syndrome (DS). In conclusion, the main problem in managing patients with APL is still the high early death rate.