Introduction
Fanconi’s anaemia is the most common cause of inherited bone marrow failure. Patients have a predisposition for malignancies, particularly an increased risk of head and neck malignancy. The prevalence of head and neck malignancy has not yet been examined in an Irish cohort. We therefore proposed to examine the rate of malignancy in our patient population, which attend the largest haematology and head and neck cancer oncology units in Ireland.
Methods
Following institutional ethical approval, a retrospective chart review was performed of all Fanconi’s anaemia patients. We collected demographic data, rate of bone marrow transplant, follow up with otolaryngology, incidence of premalignant lesions, and incidence of head and neck cancer.
Results
There were 14 patients with Fanconi’s anaemia. Eleven patients had undergone bone marrow transplantation. Ten patients have been seen by otolaryngology in our institution, with one followed at another hospital. 20% of patients are not followed by otolaryngology. Seven patients were diagnosed with an oral premalignant lesion (50%). One patient developed squamous cell carcinoma of the oropharynx, which gives a rate of malignancy of 7%.
Conclusion
This review describes the largest cohort of FA patients in Ireland. In line with international clinical care guidelines, we recommend that all Fanconi’s patient be followed with a head and neck surgeon for appropriate surveillance of their upper airway.