Abstract

Cooley's anemia, 1 or thalassemia, 2 is a rare but well known disease. This fatal disorder is characterized by a chronic, progressive, hypochromic and microcytic type of anemia, peripheral erythroblastosis, increased number of target and oval red blood cells, decreased fragility of erythrocytes, splenomegaly, deposition of pigment in the viscera and changes in the bones. The condition was early noted to have a familial incidence and to be virtually restricted to the Mediterranean peoples. In 1937, Angelini (quoted from Wintrobe and associates 3 ) observed that in some instances the erythrocytes of apparently healthy parents and of siblings of patients suffering from this anemia showed decreased fragility. Caminopetros 4 independently confirmed this observation, finding that 22 of 30 parents tested showed reduced fragility. He also reported slight changes in the bones in some parents. A few years later, Wintrobe, Matthews, Pollack and Dobyns 3 described the occurrence in several Italian families