Studies on thalassemia. I. An extracorpuscular defect in thalassemia major. II. The effects of splenectomy in thalassemia major with an associated acquired hemolytic anemia.

Normal red blood cells, when transfused to patients with thalassemia, have been shown to have a normal life span (1-4). It has also been demonstrated that transfused erythrocytes from donors with thalassemia major rapidly disappear from the circulation of normal recipients (2, 4). This evidence supports the contention that the hemolytic component of the anemia in thalassemia is due to an intracorpuscular defect, as is the case in congenital spherocytic anemia (5) and in sickle cell anemia (6). We have had the opportunity to observe repeatedly approximately 20 children with thalassemia major during the past five years. All of these subjects have required blood transfusions to alleviate such recurring symptoms as listlessness, irritability, anorexia, fatigue, palpitations, weakness or dizziness. It was evident after review of the transfusion records that many of these children were receiving 500 cc. supplements of blood, or the equivalent in packed erythrocytes, at very short intervals, i.e., every ten days or every 25 days. Since (a) prior to transfusion, hemoglobin and hematocrit levels in any one patient were fairly constant (and definitely not rising over a period of several years), and since (b) there was no external loss of blood, it seemed logical to assume that the transfused blood was not surviving the expected