Thalassemia is a hereditary hemolytic disease characterized by abnormal hemoglobin (Hb) production; the severity of the disease is based on the specific globin chain mutation or deletion. Blood transfusions can help to treat the symptoms for several forms of thalassemia, and many patients with thalassemia can expect to have normal life expectancies. Although transfusion guidelines exist for patients with β-thalassemia, a recent survey conducted of 11 thalassemia treatment centers in California, Washington, Nevada, and Arizona found that over half of the patients had α-thalassemia (394/717, 55%), while 44% (314/717) had β-thalassemia (314/717). Almost 35% of patients were transfusion dependent, requiring 8 or more transfusions in the past year; 56% did not receive transfusions, and 9% were intermittently transfused. Based on the survey, 83% of patients were Asian. Transfusion policies varied by center, as did the prescribed Hb goal; seven centers transfused patients when Hb dropped below 7 g/dL. Almost 17% of patients were alloimmunized despite policies to match for Rh and Kell antigens; some centers extended matching to Kidd, Duffy, and S/s antigens. Iron overload was identified as the biggest challenge for treatment centers, followed by a lack of clear clinical practice guidelines.
