ACUTE PROMYELOCYTIC LEUKEMIA IN CHILDREN - EXPERIENCE OF THE ITALIAN-PEDIATRIC-HEMATOLOGY-AND-ONCOLOGY-GROUP (AIEOP)

Scanty information is available on acute promyelocytic leukemia (APL) in children, and whether differences are present with respect to the a dult form. The experience of the Italian Pediatric Hematology and Onco logy Group (AIEOP) will be presented with respect to the following asp ects: 1. Incidence of APL. The incidence of APL is generally considere d to account for 3-9% of acute myelogenous leukemia (AML) in children and approximately 10-15% in adults. Recently a single Italian pediatri c institution reported that APL constituted one third of observed acut e nonlymphocytic leukemia (AnLL) cases. Data from the AIEOP cooperativ e study group have confirmed that APL in Italy is more frequently obse rved in children as compared to other countries. Environmental and/or genetic factors should be considered to explain such differences. 2. D iagnosis of M3v. The clinical and biological features of the largest s eries of childhood M3v will be presented and the problems encountered in the proper separation of 'classic' M3 and M3v in children will be d iscussed. 3. Clinical Aspects. The clinical features of the APL patien ts enrolled in the AIEOP study groups since 1989, will be presented wi th emphasis on the recent experience with the use of all-trans retinoi c acid. 4. Analysis of PML/RAR alpha Fusion Transcripts. An RT-PCR ana lysis of 32 pediatric APL cases from cryopreserved bone marrow samples has been performed. It is concluded that APL in children did not diff er significantly from the adult form, with the exception of a higher i ncidence of PML bcr3 breakpoint.