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Clinical and hematological features of hemoglobin SC disease in Rio de Janeiro, Brazil.

1. Thirty-two patients with hemoglobin SC (Hb SC) disease from Rio de Janeiro, Brazil, are described. Mean patient age at the time of study was 20 years, and average age at onset nine years. 2. The main complaint at presentation was bone or joint pains. Symptoms involved bone or joint pains in 72% of the patients, while 59% presented hepatomegaly and 50% splenomegaly. Mean hemoglobin level was 10.6 g/dl, erythrocyte count, 3.9 X 10(6)/mm3, and serum bilirubin, 1.4 g/dl. Reticulocytes and Hb F were 3.0% and 2.2%, respectively. 3. The characteristics of Hb SC disease observed in the present study did not differ significantly from those of other series studied elsewhere. This relative homogeneity of data is probably related to the physiopathological mechanism that promotes sickling in this condition.