ANEMIA DISERITROPOIÉTICA CONGÊNITA TIPO II

Introduction: Congenital Deserythropoietic Anemia (CDA) is a heterogeneous group of diseases, characterized mainly by dyseritropoiesis and ineffective erythropoiesis. The diagnosis is exclusionary, that is, it is necessary to rule out all other forms and causes of congenital or acquired dyseritropoiesis to be sure of its diagnosis. In addition to anemia (mild to moderate), patients may have jaundice, splenomegaly, and deficiency in hematopoietic response (a determining factor of anemia intensity). Type II CDA, the most common form of congenital dyseritropoetic anemia. Most are diagnosed in childhood or eventually in adulthood; Few cases are diagnosed at birth. Many patients are often transfused. Objective: This article aims to present a case of a patient diagnosed at 18 months of age with type II dyseritropoetic anemia who underwent frequent transfusions, using depherosirox (iron chelator) and folic acid. Materials and methods: The research is descriptive. Data were obtained through analysis of the patient's medical record. Conclusion: CDA is a rare disease that is difficult to diagnose. The most conventional treatment of choice is program or intermittent blood transfusion associated with the use of iron chelator.