title: Influence of alpha-thalassemia trait on spleen function in sickle cell anemia patients with high HbF.
abstract: Spleen function was studied in a group of 20 Kuwaiti SS patients (aged 2-12 years), using 99mTc-labeled tin colloid scintigraphy. They were screened for the alpha-thalassemia determinants which are prevalent in the Arabian Peninsula [-alpha (3.7 kb) deletion, alpha2-globin gene polyadenylation signal (AATAAA => AATAAG) mutation, and 5' IVS-I splice junction pentanucleotide (GAGGTGAGG => GAGG) deletion] with a combination of polymerase chain reaction and allele-specific oligonucleotide (ASO) hybridization techniques. The patients were divided into three groups depending on the result of their colloid uptake. Group I consisted of 7 patients (35.0%) with normally visualized spleens, Group II consisted of 5 (25.0%) with partial visualization, and in Group III there were 8 (40.0%) in whom the spleen was not visualized at all. The significant distinguishing features among those in Groups I and III were mean corpuscular volumes (MCVs) of 74.1 +/- 5.1 and 90.1 +/- 6.6 fl (P<0.0001) and mean corpuscular hemoglobins (MCHs) of 22.4 +/- 2.7 and 27.5 +/- 4.0 pg (P<0.05), respectively. The overall frequency of alpha-thalassemia determinants in the study was 35.0%; however, the frequencies in Groups I, II, and III were 57.1, 30.0, and 18.8%, respectively. alpha-Thalassemia trait, therefore, appears to be associated with normal splenic function in these patients.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Kuwait University.
authors.forename: A D
authors.initials: AD
authors.lastname: Adekile
authors.forename: M
authors.initials: M
authors.lastname: Tuli
authors.forename: M Z
authors.initials: MZ
authors.lastname: Haider
authors.forename: K
authors.initials: K
authors.lastname: Al-Zaabi
authors.forename: S
authors.initials: S
authors.lastname: Mohannadi
authors.forename: A
authors.initials: A
authors.lastname: Owunwanne
mesh_terms: D000367:Age Factors; D000483:Alleles; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001424:Bacterial Infections / Q000150:complications; D001609:Beta-Globulins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003102:Colloids; D005260:Female; D005293:Ferritins / Q000097:blood; D005319:Fetal Hemoglobin / Q000378:metabolism*; D005838:Genotype; D005914:Globins / Q000235:genetics; D006239:Haplotypes; D006400:Hematocrit; D006760:Hospitalization; D006801:Humans; D007730:Kuwait; D008297:Male; D009693:Nucleic Acid Hybridization; D009841:Oligonucleotides; D016133:Polymerase Chain Reaction; D011877:Radionuclide Imaging; D013154:Spleen / Q000000981:diagnostic imaging / Q000503:physiopathology*; D013667:Technetium; D014004:Tin Radioisotopes; D017085:alpha-Thalassemia / Q000097:blood / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*
chemical_list: D001609:Beta-Globulins; D003102:Colloids; D009841:Oligonucleotides; D014004:Tin Radioisotopes; D013667:Technetium; D005914:Globins; D005293:Ferritins; D005319:Fetal Hemoglobin