[Epidemiology and course of splenomegaly in children and adolescents with sickle cell disease in Senegal]

Abstract

Splenomegaly is common in sickle cell disease (SCD) and can lead to complications. In order to evaluate its prevalence and progression in Senegalese children ad adolescents with SCD, we analyzed the records of all patients followed-up at Albert-Royer Children's Hospital in Dakar, Senegal, from January 1991 to December 2005. Age, clinical course specifying size of the spleen beyond the costal margin, and disease progression were the main data recorded. We included 698 patients (94.6% SS, 4.4% SC and 1% S-beta-thalassemia). The subjects' mean age when included in the cohort was 6 years and 11 months. Splenomegaly was observed in 122 patients (17.5 %), measuring 1-17 cm beyond the costal margin (mean, 4.7 cm). Splenomegaly was more frequent in SC patients (32.3 %) compared to SS patients (16.5 %, p=0.025). The frequency was greater in infants aged 0-12 months (25.6 %) and increased from the 1- to 5-year-old age group to the 6- to 10-year-old, before it decreased further. The course of splenomegaly was marked by subsequent reduction in 17.3 % of cases, total reversal in 43.4 %, and no size variation in 39.3 %. Acute splenic sequestration occurred in 3 SS cases aged 27, 29, and 32 months and was managed with blood transfusion. Seven patients (6 SS and 1 SC) aged 4-13 years presented hypersplenism. Splenectomy was performed in 4 of these cases, while the complication reversed after 2 to 3 blood transfusions in the 3 remaining cases. The prevalence of splenomegaly is relatively low in Senegalese children with SCD. The risk of complications requires monitoring the spleen and teaching parents palpation of the baby's abdomen for early management of splenic sequestration.