Qasrawi Ayman, Gomes Victor, Chacko Charles Andrew, Mansour Akila, Kesler Melissa, Arora Ranjana, Wei Sainan, Ramlal Reshma, Munker Reinhold
Department of Medicine, University of Kentucky, Lexington, KY, USA.
Federal University of Ceara, Fortaleza, Brazil.
Leuk Res. 2020 Feb;89:106301. doi: 10.1016/j.leukres.2020.106301. Epub 2020 Jan 17.
Acute undifferentiated leukemia (AUL) is rare and defined by the absence of bona fide myeloid and lymphoid markers. Little is known about its incidence, survival and optimal management in the recent time period. Based on a case observed in our clinic, we queried the Surveillance, Epidemiology, and End Results database between 2000 and 2016. A total of 1,888 cases of AUL were diagnosed (1.34 per million person-years). The incidence of AUL has significantly decreased over time. Compared to other acute leukemias, patients with AUL have the highest median age (74 years); in contrast to acute myeloid leukemia (AML, 65) and acute lymphoblastic leukemia (ALL, 12). Excluding patients with preexisting malignancies, 1,444 patients with AUL were analyzed for survival. Only 35% of AUL patients had received chemotherapy. Comparatively, 94% of ALL and 71% of AML cases received chemotherapy. Among AUL patients who received chemotherapy, the median survival was 12 months as opposed to 1 month in the group who did not receive chemotherapy (or unknown status). Among adults, AUL patients had the worst prognosis, with a median overall survival (OS) of 9 months, compared to 27 months in ALL and 13 months in AML. Among children, the median OS was superior for all three groups of leukemias, the OS of AUL patients being better than in AML and very similar to ALL. On multivariate analysis, older age and time period were associated with worse outcome. We describe here the largest series of cases with AUL published to date.
急性未分化白血病(AUL)较为罕见,其定义为缺乏真正的髓系和淋巴系标志物。近期对其发病率、生存率及最佳治疗方案了解甚少。基于我们诊所观察到的一例病例,我们查询了2000年至2016年的监测、流行病学和最终结果数据库。共诊断出1888例AUL病例(每百万人口年发病率为1.34例)。AUL的发病率随时间显著下降。与其他急性白血病相比,AUL患者的中位年龄最高(74岁);与急性髓系白血病(AML,65岁)和急性淋巴细胞白血病(ALL,12岁)形成对比。排除既往有恶性肿瘤的患者后,对1444例AUL患者进行了生存分析。只有35%的AUL患者接受了化疗。相比之下,94%的ALL患者和71%的AML患者接受了化疗。在接受化疗的AUL患者中,中位生存期为12个月,而未接受化疗(或情况不明)的患者组中位生存期为1个月。在成人中,AUL患者的预后最差,中位总生存期(OS)为9个月,而ALL患者为27个月,AML患者为13个月。在儿童中,三组白血病的中位OS均较好,AUL患者的OS优于AML患者,与ALL患者非常相似。多因素分析显示,年龄较大和时间段与较差的预后相关。我们在此描述了迄今为止发表的最大系列的AUL病例。