Abstract
Since Pauling (1949) mentioned that abnormalities of hemoglobin formation may be genetically determined, there has been a tremendous impetus for the study of both normal and abnormal hemoglobin patterns. The medical literature has become voluminous on the various hemopathic syndromes, although little is to be found in the dermatologic journals. Thalassemia, or Mediterranean anemia, is being seen and recognized with increased frequency by dermatologists; hence a review of the syndrome seems indicated. Cooley and Lee (1925) first described a disorder occurring in children of Mediterranean descent and characterized by anemia, hepatosplenomegaly, Mongoloid appearance, and discoloration of the skin and sclerae. Laboratory examinations showed there was bile in the urine and increased resistance of the patient's erythrocytes to hypotonic solutions. In addition, leukocytosis and nucleated red blood cells, chiefly of the normoblastic variety, were present in the blood. Roentgenograms showed enlargement of the cranial and