Abstract
Abstract
Background:
The spleen is one of the first organs affected by sickle-cell disease (SCD) due to frequent sickling. Autosplenectomy usually occurs during adolescence in many places worldwide; patients in India frequently have a distinct disease history. This study aimed to investigate splenic morphology and its association with clinical manifestations and hematological parameters in patients with homozygous SCD.
Materials and Methods:
In a prospective cohort study, 110 individuals with homozygous SCD participated at Kasturba Hospital in Sevagram, Wardha, India. The presence of the spleen, its size, morphology, and complications were evaluated using ultrasonography. Quantification of hemoglobin (Hb) was done using high-performance liquid chromatography. The clinical manifestations and hematological parameters were assessed according to the standard protocols.
Results:
Vaso-occlusive crisis was the most common clinical manifestation (70.9%), followed by cholelithiasis (10.91%), avascular necrosis of bone (7.27%), and acute chest syndrome (5.45%). The mean spleen size was 13.22 ± 2.46 cm. Abnormal spleen sizes were observed in 49.09% of patients. Fetal Hb (HbF) levels showed a significant positive correlation with spleen size and an inverse relationship with clinical manifestations. white blood cell count, platelet count, and mean corpuscular volume were significantly associated with splenomegaly.
Conclusion:
Nearly half of the patients with homozygous SCD exhibited abnormal spleen sizes, with splenomegaly being more common than autosplenectomy. Elevated HbF levels appeared to protect the splenic structure and reduce the incidence of clinical manifestations. Hematological parameters were correlated with splenomegaly. Monitoring HbF and spleen size may serve as valuable indicators of disease severity and management in these patients.