title: Oral manifestations compatible with chronic graft-versus-host disease in patients with Fanconi anemia.
abstract: Fanconi anemia (FA) is a genetic disease that is characterized by several congenital abnormalities and progressive bone marrow failure and is associated with an increased susceptibility to malignant disorders. Currently, the only potential cure for hematological disorders is hematopoietic stem cell transplantation (HSCT). However, 1 of the most common complications after HSCT is the development of oral chronic graft-versus-host disease (cGVHD), which is also a risk factor for the development of cancer, particularly oral squamous cell carcinoma. Therefore, the purpose of this study was to describe the prevalence and characteristics of oral manifestations compatible with cGVHD in patients diagnosed with FA according to the National Institutes of Health (NIH) consensus criteria. A total of 96 patients (51 females, 45 males; median age, 16 years) with FA, who were in medical follow-up after HSCT at the outpatient clinic of the bone marrow transplantation unit (Hospital de Clínicas from the Universidade Federal do Paraná) underwent an oral evaluation between January 2013 and December 2013. Post-HSCT periods varied from 1 to 261 months and were divided into 3 periods: immediate post-HSCT period; intermediate post-HSC period, and late post-HSCT period. Among the evaluated patients, 40 of 96 (42%) presented with oral manifestations of cGVHD, with 29 of 40 (73%) of these patients in the late post-HSCT period. NIH scale scores varied from 0 to 10, and lichenoid and hyperkeratotic lesions were the abnormalities most frequently observed (100%). Overall, a high prevalence of oral manifestations was observed for cGVHD patients with FA. These data highlight the importance of monitoring oral manifestations compatible with cGVHD to identify and treat individuals with a higher risk of developing oral cancer.
authors.affiliation: Graduate Program in Dentistry, Department of Stomatology, Universidade Federal do Paraná, Curitiba, Paraná, Brazil. Electronic address: lauragrein@hotmail.com.
authors.forename: Laura
authors.initials: L
authors.lastname: Grein Cavalcanti
authors.affiliation: Graduate Program in Dentistry, Department of Stomatology, Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
authors.forename: Renata L
authors.initials: RL
authors.lastname: Fuentes Araújo
authors.affiliation: Bone Marrow Transplantation Service, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
authors.forename: Carmem
authors.initials: C
authors.lastname: Bonfim
authors.affiliation: Department of Stomatology, Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
authors.forename: Cassius C
authors.initials: CC
authors.lastname: Torres-Pereira
mesh_terms: D000293:Adolescent; D000328:Adult; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002294:Carcinoma, Squamous Cell / Q000517:prevention & control; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D036101:Cord Blood Stem Cell Transplantation / Q000009:adverse effects*; D003430:Cross-Sectional Studies; D005199:Fanconi Anemia / Q000473:pathology / Q000628:therapy*; D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D006801:Humans; D008297:Male; D009055:Mouth / Q000276:immunology / Q000473:pathology*; D009062:Mouth Neoplasms / Q000517:prevention & control; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D014019:Tissue Donors; D014184:Transplantation, Homologous
keywords: Bone marrow transplantation; Consensus Development Conference; Fanconi anemia; Graft-versus-host disease; Mouth; Mucosa; National Institutes of Health