hematology and oncology
Thalassemias
Introduction
Thalassemias are a group of inherited microcytic, hemolytic anemias characterized by defective hemoglobin synthesis. Alpha-thalassemia is particularly common among people with African, Mediterranean, or Southeast Asian ancestry. Beta-thalassemia is more common among people with Mediterranean, Middle Eastern, Southeast Asian, or Indian ancestry. Symptoms and signs result from anemia, hemolysis, splenomegaly, bone marrow hyperplasia, and, if there have been multiple transfusions, iron overload. Diagnosis is based on genetic tests and quantitative hemoglobin analysis. Treatment for severe forms may include transfusion, splenectomy, chelation, and stem cell transplantation. (See also Overview of Hemolytic Anemia .) 