Torres-Pereira Cassius Carvalho, Stramandinoli-Zanicotti Roberta Targa, Amenábar José Miguel, Sassi Laurindo Moacir, Galbiatti Pedruzzi Paola Andrea, Piazzetta Cleto M, Bonfim Carmem
Stomatology Department Professor, Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
Spec Care Dentist. 2014 Jul-Aug;34(4):212-5. doi: 10.1111/scd.12058. Epub 2013 Nov 26.
Fanconi Anemia patients are a high risk group for solid and hematologic malignancies. The risk seems to be influenced by age, chronic graft versus host disease and immunosuppressive drug regimens. Reports of oral malignant transformation in Fanconi Anemia after hematopoietic stem cell transplantation (HSCT) are increasing probably because of longer survival rates. This is the report of an 18- and her 28-year old sister who developed a post-HSCT oral squamous cell carcinoma. There were significant differences regarding time to malignant transformation, marrow donor characteristics and graft versus host disease evolution and treatment. The report reinforce the need for a routine head and neck screening for cancer in this particular syndrome and suggest that familial history should also be considered in Fanconi anemia patients at risk for oral malignancy after HSCT.
范可尼贫血患者是实体瘤和血液系统恶性肿瘤的高危人群。这种风险似乎受年龄、慢性移植物抗宿主病和免疫抑制药物治疗方案的影响。造血干细胞移植(HSCT)后范可尼贫血患者发生口腔恶性转化的报告越来越多,这可能是由于生存率提高所致。本文报告了一名18岁及其28岁的姐姐,她们在HSCT后发生了口腔鳞状细胞癌。在恶性转化时间、骨髓供体特征、移植物抗宿主病的进展和治疗方面存在显著差异。该报告强调了对这一特殊综合征患者进行常规头颈部癌症筛查的必要性,并建议对于HSCT后有口腔恶性肿瘤风险的范可尼贫血患者,也应考虑家族史。