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.

Long-term Survival, Organ Function, and Malignancy after Hematopoietic Stem Cell Transplantation for Fanconi Anemia

“…A better understanding of GVHD biology with incorporation of novel GVHD prophylaxis treatments may lower GVHD rates from what is observed with standard calcineurin inhibitor regimens [27]. The data also support aggressive surveillance for early detection of oral SCC in all long-term FA survivors [15,26,[28][29][30][31]. Frequent surveillance using noninvasive methods for screening [28] may prevent some of the deaths associated with SCC, as the treatment options in FA patients are limited and complications related to chemotherapy and radiotherapy are frequent [26,29,[32][33][34].…”

Two enemies, one fight: An update of oral cancer in patients with Fanconi anemia

“…Whereas GvHD is one of the main potential risk factors for the development of oral cancer, [53][54][55][56] there is only 1 study describing the oral manifestations of chronic GvHD in people with FA. 57 Those investigators evaluated 96 individuals with FA who underwent allogenic HSCT and found that 40 (42%) presented with chronic GvHD, mainly in the tongue, buccal mucosa, and hard palate. A high prevalence of lichenoid and hyperkeratotic lesions was observed, mainly in the late post-HSCT period, reinforcing the importance of constant surveillance.…”

“…). Whereas GvHD is one of the main potential risk factors for the development of oral cancer, there is only 1 study describing the oral manifestations of chronic GvHD in people with FA . Those investigators evaluated 96 individuals with FA who underwent allogenic HSCT and found that 40 (42%) presented with chronic GvHD, mainly in the tongue, buccal mucosa, and hard palate.…”

Risk Stratification of Oral Potentially Malignant Disorders in Fanconi Anemia Patients Using Autofluorescence Imaging and Cytology-On-A Chip Assay

“…The cumulative incidence of OSCC in FA patients who had HSCT is estimated to be almost 100% by 45 years of age, whereas FA patients without HSCT have half of this risk [9] , [14] . Increased risk for OSCC in FA patients with HSCT is attributed to the conditioning regimen and the occurrence of chronic graft-versus-host disease (cGVHD) in the oral cavity but may also be a function of increased longevity in FA patients, allowing time to develop OSCC [9] , [15] .…”