SPLENIC FUNCTION IN OMANI CHILDREN WITH SICKLE CELL DISEASE: Correlation with Severity Index, Hemoglobin Phenotype, Iron Status, and α -Thalassemia Trait

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).

Coinheritance of α-Thalassemia Decreases the Risk of Cerebrovascular Disease in a Cohort of Children with Sickle Cell Anemia

“…This is probably due to the reduction in the hemolytic rate (7,10,42), irreversibly sickled cells (6,14), as well as a possible decrease in inflammatory process. Also in concurrence with other studies, the mean levels of MCV (6)(7)(8)10,12,13,15,16,23,34,38,41,43,44) and MCH (6,8,15,23,43,44) were significantly lower in subjects with α-thal. This is due to the reduction in the synthesis of α chains (45).…”

“…Coexistence of α-thal apparently increases Hb A 2 levels in sickle cell anemia (8,13,15) and does not influence Hb F levels (9,15,16). Studies have shown that α-thal is associated with a reduced prevalence of cerebral stroke (6,20) and leg ulcers (6,21) and preserves splenic function (22,23), perhaps because of a reduction in hemolysis and improved PCV levels prejudicial effects are seen in complications that are perhaps more dependent on blood viscosity, such as painful episodes (15,16,18,24) and osteonecrosis (24,25). It has also been suggested that the coexistence of α-thal would prolong the life expectancy of patients with sickle cell anemia (26,27).…”

Jaw Osteomyelitis as a Complication of Sickle Cell Anaemia in Three Omani Patients: Case reports and literature review

“…It is possible that the low number of SCJO cases may be due to the prevalence of preserved splenic function in Omani patients. 15 In the largest published case series to date, Olaitan et al reported that jaw osteomyelitis occurred predominantly in men (81%) among 16 SCJO patients. 6 Other reports have also noted a higher prevalence of SCJO among men.…”

Computed Tomography Findings in the Spleen of Sickle Cell Patients in Saudi Arabia.

“…Reports from the Eastern Province of Saudi Arabia and gulf region showed that spleen function is usually preserved in patients with sickle cell anemia (SCA) who presented with levels of Hb F higher than 20% and had a high prevalence of alpha thalassemia trait. Ngo et al, 2013;Wali et al, 2002) This study was conducted to see the abnormal splenic finding among patients with sickle cell disease in Al-Ahsa, aregion in the Eastern Province of Saudi Arabia. The study documented different splenic pathologies that were found among 119 patients.…”