DIAMOND-BLACKFAN ANEMIA AND MALIGNANCY - A CASE-REPORT AND A REVIEW OF THE LITERATURE

Background. Diamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome involving the erythropoietic lineage. A preleukemic predisposition has been suggested but not proven. Methods. The development of Hodgkin's disease in a 18-year-old boy with DBA, in remission for 13 years after cessation of steroid therapy, is described. Review of the literature revealed 11 other cases of malignancy (10 hematologic) in DBA. Results. This patient, together with those described in the literature, shows that the incidence of hematological malignancy in DBA is increased (2.5% of all reported cases of DBA). Treatment was successful in three, including one after allogeneic bone marrow transplantation and our patient in whom recurrence of DBA complicated treatment. Conclusions. The incidence of hematologic malignancies in patients with DBA is increased. Treatment can be successful but may be complicated by recurrence of DBA. Bone marrow transplantation should be considered for patients with a suitable donor as part of treatment of patients with DBA and hematologic malignancy.