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

Abstract

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.

Keywords:

• Splenic enlargement
• Adults
• Homozygous sickle cell disease
• Jamaica
• Clinical profile

Acknowledgements

M.A. and M.R. designed the study; M.A. and A.W. collected and entered the data; M.A. and M.R. performed the analysis; and all three authors wrote and approved the manuscript.