Management of APL in developing countries: epidemiology, challenges and opportunities for international collaboration

Abstract

Acute promyelocytic leukemia (APL), a relatively rare hematologic malignancy, is highly curable with current treatment strategies. However, these strategies may be unavailable in countries with limited resources. A review of records in several Latin American countries revealed that approximately 30% of deaths among children and adults with APL were caused by early complications associated with the disease or its treatment. Further, APL accounts for 20% to 25% of cases of AML in these countries, consistent with the previous observation of increased incidence of APL in Latin Americans. The lack of population-based registries in developing countries has made it difficult to determine the real incidence of APL. Moreover, APL appears to have other unique epidemiologic characteristics, including association of primary APL with an increased body mass index at diagnosis and association of secondary APL with breast cancer. To facilitate the development of local capacity and implement effective treatment of APL in developing countries, the International Committee of the American Society of Hematology has assembled a working group to formulate treatment guidelines based on evidence from clinical trials results in the developed world but adapted to local resources. It is hoped that uniform treatment, careful documentation of specific outcome data, and ongoing monitoring of treatment efficacy and toxicity will improve the cure rate and provide biologic and epidemiologic information about APL in developing countries. This initial demonstration project may be joined by other countries, providing a framework for additional clinical investigation in this highly curable form of leukemia.