Continuing high early death rate in acute promyelocytic leukemia: a population-based report from the Swedish Adult Acute Leukemia Registry

Abstract

9Swedish Acute Myeloid Leukemia Group, SwedenOur 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 populationbased 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 o40 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 dehydrogenase, 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.

Characterization and prognostic features of secondary acute myeloid leukemia in a population‐based setting: A report from the Swedish Acute Leukemia Registry

“…Consolidation treatment consisted of one to three courses of combination therapy including intermediate-or high-dose Ara-C. Treatment of APL followed the national guidelines with ATRA in combination with either idarubicin or daunorubicin [13]. Palliative treatment included supportive treatment with transfusions with or without low-dose cytarabine, hydroxyurea, and/or thioguanin.…”

How to manage acute promyelocytic leukemia

“…33,95,96 Park et al 97 reported 1400 cases of APL from 1992 to 2007, the overall ED rate was 17.3%, and only a modest change in ED was observed over time. Lehmann et al 98 reported 105 cases of APL patients among whom the frequency of ED (within 30 days) actually reached 29%, a rate significantly higher than those in non-APL AML, especially evident in the elderly (460 years) population. For those EDs, 41% died from hemorrhagic complications, most of which were CNS bleeding.…”

“…Therefore, early diagnosis, and proper handling of hemorrhagic diathesis is proven to be especially important. 98 Thus, for the novo APL patients, the treatment should be further optimized. Our aim is to deliver the most timely and best treatment to each newly diagnosed APL patient, that is, to mobilize our entire human and material resources if necessary, including emergency room, intensive care unit, multi-disciplinary team of physicians and nurses, to ensure the maximum saving of lives at the onset of disease.…”

A Canadian Consensus on the Management of Newly Diagnosed and Relapsed Acute Promyelocytic Leukemia in Adults

“…Despite the use of atra therapy for almost two decades, early mortality has been reduced only modestly, as recently noted in a 2011 population-based study of the Surveillance, Epidemiology, and End Results database 11 . Contrary to reported incidence rates of early death from cooperative clinical trials (which are in the 5%-10% range) 11 , recent population-based studies have shown those rates to be much higher, ranging from 17% (in a study of the Surveillance, Epidemiology, and End Results database) to 29% in a study of the Swedish Adult Acute Leukemia Registry [11][12][13] . Consistent with those findings, the results of a Canadian study by Paulson et al in 2014 showed that the incidence of early death was 22% 14 .…”