Grein Cavalcanti Laura, Fuentes Araújo Renata L, Bonfim Carmem, Torres-Pereira Cassius C
Graduate Program in Dentistry, Department of Stomatology, Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
Graduate Program in Dentistry, Department of Stomatology, Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
Biol Blood Marrow Transplant. 2015 Feb;21(2):275-80. doi: 10.1016/j.bbmt.2014.10.009. Epub 2014 Oct 12.
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.
范可尼贫血（FA）是一种遗传性疾病，其特征为多种先天性异常和进行性骨髓衰竭，并与恶性疾病易感性增加有关。目前，血液系统疾病唯一可能的治愈方法是造血干细胞移植（HSCT）。然而，HSCT后最常见的并发症之一是口腔慢性移植物抗宿主病（cGVHD）的发生，这也是癌症尤其是口腔鳞状细胞癌发生的一个危险因素。因此，本研究的目的是根据美国国立卫生研究院（NIH）的共识标准，描述符合cGVHD的口腔表现在诊断为FA的患者中的患病率和特征。共有96例FA患者（51例女性，45例男性；中位年龄16岁），他们在骨髓移植科门诊（巴拉那联邦大学临床医院）进行HSCT后接受医学随访，于2013年1月至2013年12月接受了口腔评估。HSCT后的时间从1个月到261个月不等，分为3个时期：HSCT后即刻期；HSCT后中期，以及HSCT后期。在评估的患者中，96例中有40例（42%）出现cGVHD的口腔表现，其中40例中的29例（73%）在HSCT后期。NIH量表评分从0到10不等，苔藓样和角化过度病变是最常观察到的异常（100%）。总体而言，观察到FA的cGVHD患者口腔表现的患病率较高。这些数据突出了监测符合cGVHD的口腔表现以识别和治疗患口腔癌风险较高个体的重要性。
