Prevalence of Splenomegaly and Splenic Complications in Adults with Sickle Cell Disease and Its Relation to Fetal Hemoglobin

Background: Spleen has been found to be the earliest organ involved in sickle cell disease (SCD) patients with variable manifestations in different geographical regions. It usually undergoes autosplenectomy by adolescence but in countries like India, the course of the disease and splenic manifestations are different. And here we aim to study these differences and the relationship between spleen size and fetal hemoglobin (HbF) and various splenic complications in our patients with sickle cell disease. Materials and Methods: This is an observational study of 62 adult patients with sickle cell disease admitted in our prestigious institute in the northwestern part of India, mostly hailing from the tribal population. The clinical and ultrasonographic methods have been used to identify splenomegaly and spleen size and prevalence have been calculated. The correlation coefficient has been calculated between fetal hemoglobin, sickle hemoglobin, and spleen size. Results: The analysis done revealed that 77.4% of patients had abnormal spleen with high average HbF(14.9±5.0) values compared to those who had normal spleen(12.12±4.1). Only 2 patients were found to have no spleen and 3.3% had splenic infarct. All patients with splenomegaly had anemia, 51.6% of patients were in sickle cell crisis and 22.5% were having infections. We also found a weak but positive correlation between spleen size and HbF. Conclusion: This study revealed the persistence of the spleen, the high prevalence of splenomegaly in the Indian adult population with sickle cell disease, and higher levels of fetal hemoglobin, the exact reason for which is still a subject of speculation that needs research. But this paper provides clear evidence of different natural courses of SCD in India.