Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients.
OBJECTIVE
This study aimed to document the transition of hemoglobin (Hb) F levels from early childhood to adulthood in Kuwaiti sickle cell disease patients, investigating its relationship to sex, Hb genotype and coexistence of alpha-thalassemia trait.


METHODS
The following parameters were extracted from the patients' records: age, sex, Hb, mean corpuscular volume, mean corpuscular Hb, red blood cell count, Hb F, Hb S, Hb A(2) and alpha-globin genotype. Hb quantitation was performed with cation exchange HPLC, while alpha-globin genotype was determined by PCR.


RESULTS
Records were available for 149 patients, made up of 94 SS and 55 Sbeta(0)thal; 83 males and 66 females, aged 3 months to 60 years (mean 10.5 +/- 1.8). The mean Hb F level in the whole population was 21.5 +/- 8.1% and was not significantly different between males and females, and SS or Sbeta(0)thal. When the age groups were analyzed, the Hb F level was highest (28.9 +/- 10.9%) in those below 5 years. Indeed, patients < or =2 years had a mean level of 31.9 +/- 13.0%. There was no significant difference in the Hb F levels in SS patients with or without coexistent alpha-thal trait.


CONCLUSIONS
Kuwaiti sickle cell disease patients below 5 years of age have close to 30% Hb F and this is probably a major reason why they usually do not present before this age, unlike patients elsewhere who present within the first year of life.
Adekile
Adekunle
A
Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait. adekile@hsc.edu.kw
Al-Kandari
Mohammed
M
Haider
Mohammad
M
Rajaa
Marouf
M
D'Souza
Mark
M
Sukumaran
Jalaja
J
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis*; D005838:Genotype; D006801:Humans; D007223:Infant; D007730:Kuwait / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012737:Sex Factors; D017085:alpha-Thalassemia / Q000235:genetics
D005319:Fetal Hemoglobin