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High incidence of acute promyelocytic leukemia in children in northwest Italy, 1980–2003: a report from the Childhood Cancer Registry of Piedmont

Leukemia volume 22, pages 439–441 (2008)Cite this article

Acute myeloid leukemia (AML) represents approximately 20% of acute leukemia in children (0–14 years old)1 and is divided into subgroups based on the French–American–British (FAB) classification. Subgroups are characterized by different morphology and immunohistochemical stains of the leukemia cells, specific chromosomal aberrations, different clinical courses and responses to therapy. Acute promyelocytic leukemia (APL, M3 in the FAB classification) is a distinct subtype characterized by unique morphological and molecular characteristics of myeloblasts, with a translocation between chromosomes 15 and 17 (t(15;17)) occurring in 99% of APL cases. While there is extensive literature on the clinical features of adult APL, studies dedicated to pediatric patients are limited.2

A specific feature of APL is its remarkably high incidence observed among populations of Latin origin. Observational studies have found a higher percentage of APL cases among Hispanics in Los Angeles,3 in the Mexican Mestizo population,4 in Peru,5 in Costa Rica,6 in Spain,7 in Nicaragua,8 in Cuba,9 in Italy,10 and northwest Iran.11 However, a recent study found that Hispanics did not have greater lifetime APL incidence rates than Caucasians but that the age distribution among Hispanics was significantly different from non-Hispanic Caucasians, with greater incidence rates for children (1–19 years old) and for adults up to 44 years of age.12 It has been hypothesized that the overrepresentation of APL among Hispanics could be due to an increase in the single isoform bcr1 (one of the three sites in which PML break points can occur), rather than an equal increase in all three bcr isoforms.13

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Acknowledgements

The CCRP is supported by the Piedmont Region. This project was partly supported by the ‘Oncology Special Project’, Compagnia di San Paolo/FIRMS, and the Italian Association for Cancer Research (AIRC). The funding agencies had no role in the design of this study, data collection, analysis and interpretation of the results, or the writing of the manuscript. We thank Professor Giuseppe Basso and Dr Elena Barisone for providing cytogenetic data, and Mrs Marinella Nonnato for the careful management of the CCRP data.

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Authors and Affiliations

  1. Cancer Epidemiology Unit, Childhood Cancer Registry of Piedmont, CeRMS and CPO Piemonte, University of Turin, Turin, Italy

    M M Maule, E Dama, M L Mosso, C Magnani, G Pastore & F Merletti

  2. Department of Medical Sciences, Unit of Medical Statistics and Epidemiology, University of Eastern Piedmont, Novara, Italy

    C Magnani

  3. Division of Pediatrics, Department of Medical Sciences, University of Eastern Piedmont, Novara, Italy

    G Pastore

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  1. M M Maule
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Correspondence to M M Maule.

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Maule, M., Dama, E., Mosso, M. et al. High incidence of acute promyelocytic leukemia in children in northwest Italy, 1980–2003: a report from the Childhood Cancer Registry of Piedmont. Leukemia 22, 439–441 (2008). https://doi.org/10.1038/sj.leu.2404916

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