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Acute Leukemias

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


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.

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  1. Lo-Coco F, Ammatuna E, Montesinos P, Sanz MA . Acute promyelocytic leukemia: recent advances in diagnosis and management. Semin Oncol 2008; 35: 401–409.

    CAS  Article  Google Scholar 

  2. Wang ZY, Chen Z . Acute promyelocytic leukemia: from highly fatal to highly curable. Blood 2008; 111: 2505–2515.

    CAS  Article  Google Scholar 

  3. Fenaux P, Chastang C, Chevret S, Sanz M, Dombret H, Archimbaud E et al. A randomized comparison of all transretinoic acid (ATRA) followed by chemotherapy and ATRA plus chemotherapy and the role of maintenance therapy in newly diagnosed acute promyelocytic leukemia. The European APL Group. Blood 1999; 94: 1192–1200.

    CAS  PubMed  Google Scholar 

  4. Sanz MA, Martin G, Gonzalez M, Leon A, Rayon C, Rivas C et al. Risk-adapted treatment of acute promyelocytic leukemia with all-trans-retinoic acid and anthracycline monochemotherapy: a multicenter study by the PETHEMA group. Blood 2004; 103: 1237–1243.

    CAS  Article  Google Scholar 

  5. Lengfelder E, Haferlach C, Saussele S, Haferlach T, Schultheis B, Schnittger S et al. High dose ara-C in the treatment of newly diagnosed acute promyelocytic leukemia: long-term results of the German AMLCG. Leukemia 2009; 23: 2248–2258.

    CAS  Article  Google Scholar 

  6. Sanz MA, Montesinos P . Open issues on bleeding and thrombosis in acute promyelocytic leukemia. Thromb Res 2010; 125 (Suppl 2): S51–S54.

    Article  Google Scholar 

  7. Breccia M, Latagliata R, Cannella L, Minotti C, Meloni G, Lo-Coco F . Early hemorrhagic death before starting therapy in acute promyelocytic leukemia: association with high WBC count, late diagnosis and delayed treatment initiation. Haematologica 2010; 95: 853–854.

    Article  Google Scholar 

  8. Asou N, Adachi K, Tamura J, Kanamaru A, Kageyama S, Hiraoka A et al. All-trans retinoic acid therapy for newly diagnosed acute promyelocytic leukemia: comparison with intensive chemotherapy. The Japan Adult Leukemia Study Group (JALSG). Cancer Chemother Pharmacol 1997; 40 (Suppl): S30–S35.

    CAS  Article  Google Scholar 

  9. Fenaux P, Le Deley MC, Castaigne S, Archimbaud E, Chomienne C, Link H et al. Effect of all transretinoic acid in newly diagnosed acute promyelocytic leukemia. Results of a multicenter randomized trial. European APL 91 Group. Blood 1993; 82: 3241–3249.

    CAS  PubMed  Google Scholar 

  10. Tallman MS, Andersen JW, Schiffer CA, Appelbaum FR, Feusner JH, Ogden A et al. All-trans-retinoic acid in acute promyelocytic leukemia. N Engl J Med 1997; 337: 1021–1028.

    CAS  Article  Google Scholar 

  11. de la Serna J, Montesinos P, Vellenga E, Rayon C, Parody R, Leon A et al. Causes and prognostic factors of remission induction failure in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and idarubicin. Blood 2008; 111: 3395–3402.

    CAS  Article  Google Scholar 

  12. Di Bona E, Avvisati G, Castaman G, Luce Vegna M, De Sanctis V, Rodeghiero F et al. Early haemorrhagic morbidity and mortality during remission induction with or without all-trans retinoic acid in acute promyelocytic leukaemia. Br J Haematol 2000; 108: 689–695.

    CAS  Article  Google Scholar 

  13. De Botton S, Dombret H, Sanz M, Miguel JS, Caillot D, Zittoun R et al. Incidence, clinical features, and outcome of all trans-retinoic acid syndrome in 413 cases of newly diagnosed acute promyelocytic leukemia. The European APL Group. Blood 1998; 92: 2712–2718.

    CAS  PubMed  Google Scholar 

  14. Montesinos P, Bergua JM, Vellenga E, Rayon C, Parody R, de la Serna J et al. Differentiation syndrome in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline chemotherapy: characteristics, outcome, and prognostic factors. Blood 2009; 113: 775–783.

    CAS  Article  Google Scholar 

  15. Juliusson G, Antunovic P, Derolf A, Lehmann S, Mollgard L, Stockelberg D et al. Age and acute myeloid leukemia: real world data on decision to treat and outcomes from the Swedish Acute Leukemia Registry. Blood 2009; 113: 4179–4187.

    CAS  Article  Google Scholar 

  16. Sanz MA, Lo Coco F, Martin G, Avvisati G, Rayon C, Barbui T et al. Definition of relapse risk and role of nonanthracycline drugs for consolidation in patients with acute promyelocytic leukemia: a joint study of the PETHEMA and GIMEMA cooperative groups. Blood 2000; 96: 1247–1253.

    CAS  PubMed  Google Scholar 

  17. Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR et al. Proposed revised criteria for the classification of acute myeloid leukemia. A report of the French-American-British Cooperative Group. Ann Intern Med 1985; 103: 620–625.

    CAS  Article  Google Scholar 

  18. Matasar MJ, Ritchie EK, Consedine N, Magai C, Neugut AI . Incidence rates of acute promyelocytic leukemia among Hispanics, blacks, Asians, and non-Hispanic whites in the United States. Eur J Cancer Prev 2006; 15: 367–370.

    Article  Google Scholar 

  19. Douer D, Preston-Martin S, Chang E, Nichols PW, Watkins KJ, Levine AM . High frequency of acute promyelocytic leukemia among Latinos with acute myeloid leukemia. Blood 1996; 87: 308–313.

    CAS  PubMed  Google Scholar 

  20. Ruiz-Arguelles GJ . Promyelocytic leukemia in Mexican Mestizos. Blood 1997; 89: 348–349.

    CAS  PubMed  Google Scholar 

  21. Tomas JF, Fernandez-Ranada JM . About the increased frequency of acute promyelocytic leukemia among Latinos: the experience from a center in Spain. Blood 1996; 88: 2357–2358.

    CAS  PubMed  Google Scholar 

  22. Jacomo RH, Melo RA, Souto FR, de Mattos ER, de Oliveira CT, Fagundes EM et al. Clinical features and outcomes of 134 Brazilians with acute promyelocytic leukemia who received ATRA and anthracyclines. Haematologica 2007; 92: 1431–1432.

    Article  Google Scholar 

  23. Mandelli F, Latagliata R, Avvisati G, Fazi P, Rodeghiero F, Leoni F et al. Treatment of elderly patients (&gt; or=60 years) with newly diagnosed acute promyelocytic leukemia. Results of the Italian multicenter group GIMEMA with ATRA and idarubicin (AIDA) protocols. Leukemia 2003; 17: 1085–1090.

    CAS  Article  Google Scholar 

  24. Yanada M, Matsushita T, Asou N, Kishimoto Y, Tsuzuki M, Maeda Y et al. Severe hemorrhagic complications during remission induction therapy for acute promyelocytic leukemia: incidence, risk factors, and influence on outcome. Eur J Haematol 2007; 78: 213–219.

    Article  Google Scholar 

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Correspondence to S Lehmann.

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Lehmann, S., Ravn, A., Carlsson, L. et al. Continuing high early death rate in acute promyelocytic leukemia: a population-based report from the Swedish Adult Acute Leukemia Registry. Leukemia 25, 1128–1134 (2011).

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  • acute promyelocytic leukemia
  • incidence
  • early mortality
  • hemorrhagic death
  • population based

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