ORAL HEALTH ADEQUACY IN PATIENTS WITH BLACKFAN-DIAMOND ANEMIA

Blackfan-Diamond anemia is a rare congenital disease and there are possible treatments: Hematopoietic stem cell transplantation, steroids, and platelets and red blood cells transfusion. A 25-year-old male diagnosed with Blackfan-Diamond anemia, with insulin-dependent diabetes, developed medullary failure and underwent supportive treatment with monthly red blood cells transfusions. As a result of the multiple transfusions, he presented iron overload. At the oral physical examination, there were caries lesions on all teeth, generalized biofilm accumulation and presence of root remains. Considering the patient’s systemic conditions and the severity of oral health, it was decided to perform multiple exodontia in an operating room. For that, pre, trans, and post-procedure platelet transfusion was required, as well as other maneuvers for local hemostasis. Root remains or teeth with extensive coronary destruction were extracted. There were no intercurrences and, after the patient’s recovery, the treatment was continued in an outpatient environment.