Clinical and biochemical manifestations of severe sickle cell anemia in adult patients in steady state in Ile-Ife, Nigeria

Abstract

Background: There are documented established markers (indices) of disease severity in patients with sickle cell anemia (SCA) and they determine the course of the disease. This study investigated the pattern and prevalence of some of these markers of disease severity in adult patients with SCA in steady state attending the hematology clinic of a federal tertiary teaching hospital in Ile-Ife, Nigeria.

Materials and Methods: This was a descriptive study. Basic demographic data and relevant clinical information was obtained using a well-structured questionnaire and the case files (hospital records) of 50 consecutive SCA (HbSS) patients.

Results: The study group comprised of 21(42%) males and 29(58%) females. The age range was 18–45years with a mean(±SD) of 27.6±7.607. Hepatomegaly (64%), frequent episodes of vaso-occlusive crisis, that is, ≥ 3 episodes per year(30%) and Dactylitis in infanthood(26%) were the most common clinical parameters identified in these patients while a high serum total bilirubin of > 51μmol/L(26%) was the most common laboratory parameter seen in these patients.

Conclusion: Markers of disease severity were identified in the patients with SCA in
this study. The presence of these markers in an SCA patient connotes severe disease and they determine the course of the disease. Therefore, there is a need to pay more attention to these patients by following them up more closely.

Keywords: disease severity, markers, sickle cell anemia, steady state, adults, patients