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Sickle cell anemia: a significant potential cause of pulmonary hypertension in Brazil.

The distribution of the HbS gene in Brazil is quite heterogeneous and depends on the racial composition of the population in a given region. The prevalence of heterozygotes is higher in the northern and northeastern regions of the country (6-10%) and lower in the southeastern and southern regions (2-3%). Sickle cell anemia is commonly accompanied by infectious, hemolytic, and veno-occlusive events. Pulmonary complications account for 20-30% of all sickle cell anemia-related deaths and primarily include acute chest syndrome and pulmonary hypertension (PH).