Oral manifestations in congenital hypoplastic anemia (Diamond-Blackfan anemia): clinical report.

Congenital hypoplastic anemia is a rare disease characterized by a normochromic, normocytic anemia, a deficiency of erythroblasts in the marrow, and normal leukocyte and platelet counts. The disease usually is treated successfully with corticosteroid therapy; however, some patients become refractory to corticosteroids and must receive washed, packed red blood cell transfusions. The management and unique dental findings of a 15-yearold patient with congenital hypoplastic anemia are described. ^.ongenital hypoplastic anemia is a rare disease developing in early childhood. This disease is characterized by a normochromic, normocytic anemia, a deficiency of erythroblasts in the bone marrow, and normal or near-normal leukocyte and platelet counts." Diagnosis is based on both blood and marrow analyses. Although the etiology and pathogenesis of congenital hypoplastic anemia are still obscure, most children with this disease have long-term survival and the possibility of spontaneous remission exists.Diamond and Blackfan 1938,were the first physicians to describe congenital hypoplastic anemia as a separate entity. The first successful treatment of this disease with corticosteroids was reported in 1951. Although treatment is usually successful with continual low-dose corticosteroid therapy, some patients become refractory to corticosteroids and must receive washed, packed red blood cell transfusions every four to eight weeks. Effects of extended corticosteroid therapy include cushingoid fades, weight gain, and growth retardation. Complications of repeated transfusion therapy include hemosiderosis and cirrhosis. Only one report appears in the dental literature describing congenital hypoplastic anemia and its oral findings. The oral findings reported were severe gingivitis, multiple carious lesions, and poor healing of recent extraction sites. The purpose of this clinical report is to present some unusual dental findings in a patient with this disease.