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September 1956


Author Affiliations

Houston, Texas

Herrmann Hospital and Baylor University College of Medicine.

AMA Arch Intern Med. 1956;98(3):356-364. doi:10.1001/archinte.1956.00250270100014

SINCE Cooley and Lee's description of Mediterranean anemia, in 1925, a voluminous literature has accumulated on the subject. This disease entity, because of its variable manifestations, has been described and discussed under several names— Cooley's anemia, erythroblastic anemia, thalassemia, Mediterranean hemolytic disease, Mediterranean anemia, and Mediterranean hemopathic syndrome. This constitutional hereditary defect in hemopoiesis is manifest clinically as a disease whose severity may vary from exhibiting only characteristic abnormalities of the erythrocytes to being a severe anemia with hepatosplenomegaly, leg ulcers, and bone changes, with fatal termination in infancy or early childhood. The erythocytes characteristically show anisocytosis, poikilocytosis, leptocytosis, and increased resistance to hypotonic saline, with the presence of many target cells. Although this disease occurs usually in persons of Mediterranean extraction, well-documented examples of its occurrence in persons of Indian, Chinese. African, and pure English stock have been reported. Despite the variable clinical manifestations and varied racial incidence of

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