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Early death rates remain high in high-risk APL: update from the Swedish Acute Leukemia Registry 1997–2013

Leukemia volume 31, pages 1457–1459 (2017)Cite this article

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With the introduction of all-trans-retinoic acid (ATRA) and arsenic, the outcome of patients with acute promyelocytic leukemia (APL) has improved considerably over the last decades.1 As treatment resistance significantly has been reduced due to new and more effective treatments, early deaths (EDs), mainly due to APL-specific coagulopathy, emerge as a major threat to APL patients. We previously reported an ED rate of almost 30% in the population-based Swedish Acute Leukemia-Registry in 2009.2, 3 This has been followed by a number of epidemiological studies showing ED rates considerably surpassing those of clinical trials.4, 5, 6, 7 This is likely to be due to the fact that APL cohorts within clinical trials are highly selected for patients with a lower risk of ED and, thus, in order to assess the true ED rates, analyses from population-based registries are needed. As a result of the report from the Swedish Registry in 2009, the awareness of the threat of ED was significantly increased in the Swedish hematology community and information stressing the importance to adhere to APL guidelines was spread. These already existing guidelines included immediate start of ATRA treatment at slightest suspicion of APL and vigilant supportive care measure with aggressive policies regarding fibrinogen concentrate and/or plasma and platelet transfusions. In this report we aimed to update our population-based APL cohort with data from 2007 to 2013, encompassing the period of substantially increased awareness and vigilance concerning ED in APL and thus evaluating the effect of this increased awareness of the risk of ED.

The mean incidence of APL in Sweden during the study period from 1997 to 2013 was 11.7 new cases per year (range 7–18). In total, 195 patients were diagnosed nationwide of which 107 (55%) were female (Table 1). The median follow-up was 6.0 years. In 94% of the patients, the disease was molecularly defined either by cytogenetics, fluorescence in situ hybridization or reverse transcriptase-PCR for the PML-RARA fusion. Cases lacking molecular confirmation were reviewed morphologically and immunophenotypically to confirm APL characteristics. A substantial proportion of the newly diagnosed APL patients were elderly, with 30% being above 65 years, 20% above 70 and 14% above 75 years of age. This highlights the fact that a substantial number of APL patient are older, requiring special considerations with regard to treatment toxicity. The median age of APL patients for the whole period was 56 years, which is higher compared with any other previously reported APL cohort. This is likely to be due to the compulsory central reporting of all cancer cases nationwide to the Swedish Cancer Registry regardless of treatment intention.8

Table 1 Characteristics of APL patients in the study
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Authors and Affiliations

  1. Department of Medical Sciences, Uppsala University, Uppsala, Sweden

    S Lehmann & M Höglund

  2. Department of Medicine, Unit of Hematology, Karolinska Institute, Huddinge, Stockholm, Sweden

    S Lehmann & S Deneberg

  3. Department of Hematology, Linköping University Hospital, Linköping, Sweden

    P Antunovic

  4. Department of Hematology, Karolinska University Hospital, Solna, Stockholm, Sweden

    Å Rangert-Derolf

  5. Department of Medicine, Sahlgrenska University Hospital, Göteborg, Sweden

    H Garelius, L Möllgård & L Wennström

  6. Department of Hematology, Oncology and Radiation Physics, Skåne University Hospital, Lund, Sweden

    V Lazarevic & G Juliusson

  7. Department of Medicine, School of Health and Medical Sciences, Örebro University Hospital, Örebro, Sweden

    K Myhr-Eriksson, B Uggla & A Wahlin

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  1. S Lehmann
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Correspondence to S Lehmann.

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Lehmann, S., Deneberg, S., Antunovic, P. et al. Early death rates remain high in high-risk APL: update from the Swedish Acute Leukemia Registry 1997–2013. Leukemia 31, 1457–1459 (2017). https://doi.org/10.1038/leu.2017.71

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