Splenic enlargement in adults with homozygous sickle cell disease: the Jamaican experience

Objective: It is quite rare to find splenomegaly or its various complications in adults with homozygous sickle cell (SS) disease. Splenomegaly in adults may indicate a differential severity of disease, when compared with those who have had 'autosplenectomy'. Methods: We studied the clinical and haematological profiles of all SS patients (n = 25) in our database who have had splenomegaly in their adulthood (group 1) and compared with a random sample of age- and gender-matched persons (n = 75) who did not have splenomegaly in adulthood (group 2). Results: Group 1 persons had higher foetal haemoglobin, lower platelets and white blood cells, and greater prevalence of gall bladder disease and hospitalizations. Seven of the 25 persons in group 1 had acute splenic sequestration and the rest had hypersplenism. Conclusions: Splenic enlargement occurring during adulthood may imply lower severity of disease and clinicians should consider this diagnosis even in persons with SS genotype.