Sonographic Assessment of Splenic Manifestations in Sickle Cell Disease Patients and Its Relation to Hematological Parameters: A Cross-Sectional Study in Basra, Iraq

Cureus. 2024 Oct 24;16(10):e72322. doi: 10.7759/cureus.72322. eCollection 2024 Oct.

Abstract

Background Hemoglobinopathies, such as sickle cell disease (SCD), are inherited disorders of hemoglobin (Hb) synthesis. SCD presents with complex clinical manifestations, including anemia, painful episodes, and organ damage due to recurrent vaso-occlusion. The spleen is one of the first organs affected in SCD patients, with hyposplenism typically occurring by age five. However, older patients may show variations in splenic size, which can complicate the diagnosis. Objective This study aimed to investigate the radiological manifestations of the spleen in patients with SCD using sonographic assessment and the relationship between these manifestations and the patient's clinical and laboratory findings. Methodology This descriptive cross-sectional study was carried out at the Basra Hematology Center, which is the largest center for hematological diseases in the southern part of Iraq. It was carried out over a three-month period from May 22 to August 22, 2024. A total of 81 patients aged 15 and above, diagnosed with SCD, and attending the outpatient clinic at Basra Hematology Center in Basra city were included. Participants were identified through medical records and referrals from private outpatient clinics. Inclusion criteria ensured participants had a confirmed SCD diagnosis and met the age requirements. Exclusion criteria eliminated individuals with sickle cell trait, other hemolytic anemias, leukemia, relevant co-morbidities, or those who declined participation. After obtaining informed consent, each patient was interviewed by the research team, their blood was taken for lab tests, and then an ultrasound scan of their spleen was performed. Results Regarding the demographic characteristics of patients, age showed a statistically significant difference across the groups (p=0.017), indicating that marked splenomegaly is associated with younger individuals (mean age of 19.50 ± 4.95 years) compared to autosplenectomy, which is linked to older patients (mean age of 35.42 ± 11.84 years). Regarding radiological findings, splenic size was categorized into normal size, splenomegaly, small spleen, and autosplenectomy. Increased echogenicity is more frequently seen in the small spleen group (87.5%, p<0.001). Focal lesions, such as infarctions, were relatively uncommon and did not show significant variation across the groups. However, the presence of Gamna-Gandy bodies (GGB) was reported in a few patients and did not vary significantly among spleen status groups. The laboratory data showed notable significant differences in several key blood parameters. Hb levels were significantly lower in patients with marked splenomegaly (6.65 ± 0.21 g/dl) compared to other groups (p=0.01). White blood cell count (WBC) and platelet count (PLT) were significantly higher in the autosplenectomy group (WBC 12.29 ± 5.67 x 10³/μL; PLT 426.16 ± 222.85 x 10³/μL), both with p-values <0.001. Conclusion This study examined splenic changes in SCD patients, finding that autosplenectomy is more common in older patients, while splenomegaly is prevalent in younger ones. Increased splenic echogenicity indicated fibrosis, and patients with splenomegaly showed lower hemoglobin levels. Autosplenectomy was associated with higher WBC and PLT levels. Although elevated HbF and certain genetic factors seemed protective against splenic atrophy, these findings were not statistically significant, warranting further research.

Keywords: autosplenectomy; gamna gandy bodies; sickle cell disease (scd); spleen infarction; spleen size; ultrasound (u/s).