title: Continuing high early death rate in acute promyelocytic leukemia: a population-based report from the Swedish Adult Acute Leukemia Registry.
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.
authors.affiliation: Hematology Centre, Karolinska University Hospital, Huddinge, Stockholm, Sweden. Soren.Lehmann@ki.se
authors.forename: S
authors.initials: S
authors.lastname: Lehmann
authors.forename: A
authors.initials: A
authors.lastname: Ravn
authors.forename: L
authors.initials: L
authors.lastname: Carlsson
authors.forename: P
authors.initials: P
authors.lastname: Antunovic
authors.forename: S
authors.initials: S
authors.lastname: Deneberg
authors.forename: L
authors.initials: L
authors.lastname: Möllgård
authors.forename: A Rangert
authors.initials: AR
authors.lastname: Derolf
authors.forename: D
authors.initials: D
authors.lastname: Stockelberg
authors.forename: U
authors.initials: U
authors.lastname: Tidefelt
authors.forename: A
authors.initials: A
authors.lastname: Wahlin
authors.forename: L
authors.initials: L
authors.lastname: Wennström
authors.forename: M
authors.initials: M
authors.lastname: Höglund
authors.forename: G
authors.initials: G
authors.lastname: Juliusson
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002423:Cause of Death; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000401:mortality; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D015473:Leukemia, Promyelocytic, Acute / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D009026:Mortality / Q000639:trends; D011379:Prognosis; D012042:Registries; D012307:Risk Factors; D017678:Sex Distribution; D013548:Sweden / Q000453:epidemiology; D013997:Time Factors; D055815:Young Adult