Abstract

MACHIN GARCIA, Sergio et al. Morbimortality rates in adult patients with sickle cell disease. Rev Cubana Hematol Inmunol Hemoter [online]. 2004, vol.20, n.2, pp. 0-0. ISSN 1561-2996.

A study was performed on 397 adult patients with sickle cell anemia, who had been followed up from January 1973 to December 1997. Two hundred were females and 197 males; 305 of these patients presented with sickle cell anemia, 63 with SC hemoglobinopathy and 29 with Sb thalassemia. The mean follow-up period was 14,3 years. The yearly admission and blood transfusion mean was lower in SC hemoglobinopathy(p<0,001). Painful vasoocclusive crisis was more frequent in Sb thalassemia whereas infections often occurred in sickle cell anemia (p<0,001). One hundred four pregnancies were developed from 87 females. There were 4 maternal deaths and 10 perinatal deaths. Differences were found in hemoglobin and reticulocyte values among hemoglobinopathies (p<0,001) but no difference was seen between sexes or among age groups (18-29 y, 30-40 y and over 40 y). Fetal hemoglobin values did not show significant difference between sexes, although they were higher in sickle cell anemia. Global survival estimates were 53 years in sickle cell anemia, 59 years in SC hemoglobinopathy and 48 in Sb thalassemia (p< 0,05). The most frequent cases of death were hepatic complications, encephalic vascular attacks and infections

Keywords : sickle cell anemia; morbidity.