CHILDHOOD ACUTE PROMYELOCYTIC LEUKEMIA IN NICARAGUA

Background: The pediatric hematology-oncology service of the Managua 'La Mascota' Children's Hospital in Nicaragua began its activities in 1988. We report on unusual high incidence of acute promyelocytic leukemia (APL) observed in Nicaragua in the last three years. Patients and methods: From 1990 to 1992, 17 of 110 children with acute leukemia were diagnosed as having acute non-lymphoblastic leukemia (AnLL). The diagnosis was performed according to the FAB criteria. In two cases DNA was recovered for molecular analysis of the t(15;17). Results: Ten of 17 cases of AnLL were classified as APL. PML and RAR alpha gene rearrangements were observed in the two cases where molecular analysis was performed. Nine children received chemotherapy; one was treated with all-trans-retinoic acid (ATRA), achieving complete remission. Eight children who received chemotherapy died early of hemorrhage and/or sepsis. Discussion: In Nicaragua, APL may be much more common than is generally observed elsewhere. In recent years initial treatment of APL with ATRA has proved to reduce the incidence of early hemorrhagic deaths, and complete remissions can be achieved without marrow aplasia and thus prevent infectious complications. From that perspective APL might become a curable disease, also in developing countries where the treatment of AnLL is not usually affordable.