Hassan Inaam Bashir, Zaabi Mariam R Al, Alam Arif, Hashim Mohammed Jawad, Tallman Martin S, Kristensen Jorgen
Department of Internal Medicine, College of Medicine and Health Sciences, United Arab Emirates University, P.O. Box 17666, Al Ain, United Arab Emirates.
Department of Oncology Tawam Hospital, AlAin, United Arab Emirates.
Int J Hematol. 2017 Jul;106(1):90-98. doi: 10.1007/s12185-017-2211-7. Epub 2017 Mar 14.
Although acute promyelocytic leukemia (APL) is a curable hematologic malignancy, early death (ED) remains a significant cause of treatment failure especially in developing countries. In a retrospective data analysis of 67 adult APL patients diagnosed in United Arab Emirates we report an ED rate of 11.9% which is comparable to that reported from more developed countries. We identified the following parameters at presentation as significant predictor of increased ED: Age >40 years (P = 0.015), fever (P = 0.030), WBC count >20 × 10/L (P = 0.010), the breakpoints other than bcr1 (P = 0.043) and fibrinogen level <1.5 g/L (P = 0.025). Delay in ATRA administration beyond 24 h from admission and fibrinogen <150 mg/dL were also significant predictors of ED, but only among high-risk patients (P = 0.035 and P = 0.033, respectively). WBC count >10 × 10/L and expression of HLA-DR (P = 0.018) or CD2 (P = 0.017) were significant predictors for differentiation syndrome (DS) which was found to be a predictor of ED (P = 0.002). Reducing the APL related ED rate in centers with limited resources is feasible provided early initiation of ATRA administration and early correction of coagulopathy in high-risk patients in addition to prompt treatment of DS. To our knowledge this is the first report from the Arabian Gulf describing ED in APL.
尽管急性早幼粒细胞白血病（APL）是一种可治愈的血液系统恶性肿瘤，但早期死亡（ED）仍是治疗失败的一个重要原因，尤其是在发展中国家。在对阿拉伯联合酋长国诊断的67例成年APL患者进行的回顾性数据分析中，我们报告的早期死亡率为11.9%，这与较发达国家报告的死亡率相当。我们确定了以下就诊时的参数是早期死亡增加的显著预测因素：年龄>40岁（P = 0.015）、发热（P = 0.030）、白细胞计数>20×10⁹/L（P = 0.010）、非bcr1断点（P = 0.043）和纤维蛋白原水平<1.5 g/L（P = 0.025）。从入院起超过24小时给予全反式维甲酸（ATRA）延迟以及纤维蛋白原<150 mg/dL也是早期死亡的显著预测因素，但仅在高危患者中（分别为P = 0.035和P = 0.033）。白细胞计数>10×10⁹/L以及人类白细胞抗原-DR（HLA-DR）表达（P = 0.018）或CD2表达（P = 0.017）是分化综合征（DS）的显著预测因素，而分化综合征被发现是早期死亡的一个预测因素（P = 0.002）。在资源有限的中心降低与APL相关的早期死亡率是可行的，前提是除了及时治疗分化综合征外，还需尽早开始给予ATRA并对高危患者的凝血病进行早期纠正。据我们所知，这是阿拉伯海湾地区描述APL早期死亡的首份报告。
