Author(s): Lehmann S, Ravn A, Carlsson L, Antunovic P, Deneberg S,
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Abstract 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 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.
This article was published in Leukemia
and referenced in Biology and Medicine