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Splenic function in Omani children with sickle cell disease: correlation with severity index, hemoglobin phenotype,
Yasser A Wali1, Zakia Al-Lamki, Samir S Hussein
1Department of Child Health, Haematology/Oncology Unit, College of Medicine, Sultan Qaboos University, Al-Khod, Muscat, Oman.
Insights
Over 60% of Omani children with sickle cell disease (SCD) maintain splenic function. Factors like larger spleen size and higher HbF levels are linked to preserved spleen function in pediatric SCD patients.
Area of Science:
- Pediatric Hematology
- Medical Imaging
- Genetic Blood Disorders
Background:
- Functional asplenia is a significant complication in sickle cell disease (SCD).
- The prevalence and natural history of splenic dysfunction in Omani children with SCD remain undefined.
- Understanding splenic status is crucial for managing SCD-related complications.
Purpose of the Study:
- To determine the prevalence of functional asplenia in Omani children with SCD.
- To compare the splenic dysfunction patterns in Omani SCD patients with existing literature.
- To identify factors associated with preserved splenic function in this population.
Main Methods:
- Utilized (99m)Tc-labeled tin colloid scintigraphy to assess splenic function.
- Studied 72 Omani children (aged 4.8-16 years) with various SCD genotypes.
- Categorized patients into four groups based on colloid uptake: normal function, mild hyposplenism, severe hyposplenism, and functional asplenia.
Main Results:
- Over 60% of patients exhibited preserved splenic function (normal or mild hyposplenism).
- Functional asplenia was observed in 36% of the cohort.
- Except for HbS-beta(+) thalassemia, hyposplenism patterns were consistent across different hemoglobin phenotypes.
- Preserved spleen function correlated with larger spleen size, less clinical severity, lower MCH, higher HbF, and alpha-thalassemia trait.
Conclusions:
- A majority of Omani children with SCD retain splenic function.
- Specific factors, including genetic background and clinical presentation, influence spleen status.
- Findings provide valuable insights into the natural history of splenic dysfunction in pediatric SCD in Oman.
Abstract:
The prevalence of functional asplenia in Omani children with sickle cell disease (SCD) has not been previously defined. In this study, the authors aim to compare the natural history of splenic dysfunction in their patients to other reports. The splenic function was studied in 72 Omani patients with sickle cell disease (50 homozygous for hemoglobin S (HbS-S), 11 double heterozygotes for HbS and beta(0)-thalassemia (HbS-beta(0)-thal), 5 HbS-beta(+)-thal, 5 patients with hemoglobin S-D disease, and 1 child with hemoglobin S oman trait) aged 4.8-16 years, using (99m)Tc-labeled tin colloid scintigraphy. The study revealed 4 groups according to their colloid uptake: group I included 20 patients (28%) with normal splenic function; group II, 6 patients (8%) with mild hyposplenism; group III, 20 (28%) with severe hyposplenism; and group IV, 26 (36%) patients with functional asplenia. Overall, more than 60% of them had preserved splenic function. Except for HbS-beta(+) patients, the developmental pattern of hyposplenism was not different among the different Hb phenotypes. Factors associated with preservation of spleen function in these patients were larger splenic size (p < .01), less clinical severity (p < .05), lower MCH (p < .01), higher HbF (p < .001), and presence of alpha-thalassemia trait (p < .05).