RED-CELL MEMBRANE-PROTEIN ABNORMALITIES IN HEREDITARY SPHEROCYTOSIS IN BRAZIL

We studied 14 kindred and nine unrelated patients from southeastern Br azil with the typical form of hereditary spherocytosis. Diagnosis was made on the basis of clinical features, presence of spherocytes on the peripheral blood smears and an abnormal osmotic fragility test. By de nsitometric tracing of SDS-PAGE stained by Coomassie blue, we detected isolated deficiency of spectrin in 39% of our patients, combined spec trin and ankyrin deficiency in 13%, and deficiency of band 3 in 13%. O ne of our patients presented ankyrin deficiency without spectrin reduc tion. Our data suggest that, despite ethnic differences among the Braz ilian and European or North-American populations, these biochemical ab normalities in HS patients may be similar.