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PART I AN EXTRACORPUSCULAR DEFECT IN THALAS-SEMIA MAJORNormal 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 normal span of 120 days. Other possible explanations were that each transfusion caused a decrease in endogenous blood formation or that both of these mechanisms were involved. It was the purpose of this study to test the first assumption by measuring the survival of normal fresh blood in seven recipients with thalassemia major. METHODSThe survival of transfused erythrocytes was determined by the method of DeGowin, Sheets, and Hamilton (7). Blood from normal type 0 donors was collected aseptically 'in vacuum bottles containing "acid-citrate-dextrose" solution and was transfused to the test subjects within 36 hours. Compatibility of donor cells was assured after cross-matching by the use of the indirect Coombs test. Five of the recipients were type A and one was type AB. Anti-A sera were used in dilutions producing minimal inagglutinable cell counts. Sera capable of yielding baseline counts below 25,000 cells per cu. mm. were used. Aliquots of the selected sample of serum were kept in the deep freeze unit and were used for successive measurements during each erythrocyte life span study.One patient whose blood was type 0, MN, was transfused with 0, N cells. Lederle's anti-M serum was used for agglutination.The reliability of this method in our hands was demonstrated in normals and in patients with a variety of disorders other than acquired hemolytic anemia. Survival of transfused erythrocytes for 100 to 130 days was found in agreement with results reported by others (7-10). MATERIALThe seven patients in this series were examples of severe thalassemia major. All were of Sicilian descent and were first noted to be anemic in infancy, when blood examina...

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