STUDIES WITH INAGGLUTINABLE ERYTHROCYTE COUNTS. II. ANALYSIS OF MECHANISM OF COOLEY'S ANEMIA 1

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Abstract
Survival time of normal human erythrocytes transfused to a subject with Cooley''s anemia and Cooley''s trait was detd. by a modification of the Ashby method of differential agglutination. The survival time of Cooley''s trait cells in a normal subject and in a patient with severe Cooley''s anemia was detd. The survival time of normal cells in each instance was found to be normal. The survival of more severe Cooley''s trait cells was shorter than normal in a normal individual as well as in a patient with severe Cooley''s anemia. In each instance the curve of disappearance was linear; therefore, the cells were eliminated from circulation incident to age and not due to a random destructive force. An analysis of the ratio of the observed donor''s and recipient''s erythrocyte counts over a period of time demonstrated many periods of considerable net loss and net gain of the Cooley''s anemia''s own cells. In view of this finding and with due consideration for other laboratory and clinical findings over an observation period of 1 yr., it was thought that the periods of net loss and net gain of the cells of this subject were due to bone marrow inhibition and not due to periodic accelerated destruction of the Cooley''s anemia cells. During the periods of net deficit of the Cooley''s anemia cells the life span of these cells was considerably shorter than normal. Subjects with Cooley''s trait recovered rapidly from blood loss when used as blood donors. There seems to be little or no contraindication to the use of their blood in transfusion. Evidently the disorder in Cooley''s trait is due to an intrinsic defect in the erythrocyte which may shorten its survival in the circulation. Increased erythropoiesis, however, compensates for the loss of red cells. When the possession of this type of defective erythrocyte is combined with an insufficient rate of erythropoiesis the state of Cooley''s anemia is produced.

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