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CURRENT ISSUEITALIAN JOURNAL OF MAXILLOFACIAL SURGERY

A Journal on Maxillofacial Surgery


Official Journal of the Italian Society of Maxillofacial Surgery

 

Rivista Italiana di Chirurgia Maxillo-Facciale 2002 August;13(2):133-7

 CLINICAL CASES

Precancerous conditions and Fanconi’s anemia. A case report

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.

language: English


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