Total amount: € 0,00
Official Journal of the Italian Society of Maxillofacial Surgery
Colella G. 1, Lanza A. 1, Matarese S. M. R. 2, Nobili B. 2
1 Institute of Oral and Maxillo Facial Surgery 2 Department of Pediatry Second University of Naples, Naples
The case of an oral precancerous lesion in an 11-year-old patient, affected by Fanconi’s anemia (FA), who underwent bone marrow transplant from HLA-identical familial donor, is reported. FA is a hereditary disease with autosomal recessive transmission and a M/F ratio of 1.3/1; the incidence of the homozygotes all over the world is of 1/350,000 and in Italy of 1/20,000-1/30,000; Campania, together with Veneto, are the regions with the highest incidence of the disease, according to the data reported by the «Registro Italiano Anemia di Fanconi» (RIAF ’97). FA, together with other genetic disorders, ataxia teleangiectasia and xeroderma pigmentosum, has to be considered a precancerous disease, for its sensitivity to reactive oxygen species (ROS), and the ensuing chromosomal instability. Bone marrow transplant removes the oncologic risk for acute myeloid leukemia and myelodysplastic syndrome (MDS), but it does not alter patients’ predisposition towards other neoplasms.