Continuing high early death rate in acute promyelocytic leukemia: a population-based report from the Swedish Adult Acute Leukemia Registry
Continuing high early death rate in acute promyelocytic leukemia: a population-based report from the Swedish Adult Acute Leukemia Registry
复制标题
DOI：
10.1038/leu.2011.78
复制
发表时间：
2011-07-01
期刊：
影响因子：
11.4
通讯作者：
Juliusson, G.
中科院分区：
文献类型：
--
作者：
Lehmann, S.;Ravn, A.;Juliusson, G.
我们对急性早幼粒细胞白血病（APL）患者的了解主要基于临床试验数据，而基于人群的信息很少。我们研究了1997年至2006年诊断的APL患者在人口为基础的瑞典成人急性白血病登记。在总共3897例急性白血病病例中，3205例（82%）为非APL急性髓性白血病（AML），105例（2.7%）为APL。APL的发病率为0.145/10万人/年。诊断时的中位年龄为54岁; 62%为女性，38%为男性。在年轻APL患者中，女性占主导地位（89%的患者< 40岁）。在105例APL患者中，30例（29%）在诊断后30天内死亡（即早期死亡（艾德））（中位数为4天），28例（26%）在14天内死亡。总的来说，41%的ED是由于出血; 35%的艾德患者从未接受过全反式维甲酸治疗。艾德率随年龄增加而增加，但表现状态差者更明显。艾德还与高白色血细胞、乳酸脱氢酶、肌酐、C反应蛋白和低血小板计数有关。在非ED患者中，97%达到完全缓解，其中16%随后复发。总的来说，62%的患者在中位随访6.4年时仍然存活。我们的结论是，艾德率仍然很高，在一个AAPL人群。Leukemia（2011）25，1128-1134; doi：10.1038/leu.2011.78; 2011年4月19日在线发表
Our knowledge about acute promyelocytic leukemia (APL) patients is mainly based on data from clinical trials, whereas population-based information is scarce. We studied APL patients diagnosed between 1997 and 2006 in the population-based Swedish Adult Acute Leukemia Registry. Of a total of 3897 acute leukemia cases, 3205 (82%) had non-APL acute myeloid leukemia (AML) and 105 (2.7%) had APL. The incidence of APL was 0.145 per 100 000 inhabitants per year. The median age at the time of diagnosis was 54 years; 62% were female and 38% male. Among younger APL patients, female sex predominated (89% of patients < 40 years). Of the 105 APL patients, 30 (29%) died within 30 days (that is, early death (ED)) (median 4 days) and 28 (26%) within 14 days from diagnosis. In all, 41% of the EDs were due to hemorrhage; 35% of ED patients never received all-trans-retinoic acid treatment. ED rates increased with age but more clearly with poor performance status. ED was also associated with high white blood cells, lactate dehydro-genase, creatinine, C-reactive protein and low platelet count. Of non-ED patients, 97% achieved complete remission of which 16% subsequently relapsed. In total, 62% are still alive at 6.4 years median follow-up. We conclude that ED rates remain very high in an unselected APL population. Leukemia (2011) 25, 1128-1134; doi:10.1038/leu.2011.78; published online 19 April 2011