Sickle cell/beta-thalassemia in North Jordan.
Clinical and haematological features of 50 patients with sickle cell/beta-thalassemia (SB0 or SB+) are investigated. Total haemoglobin value was not significantly different (P greater than 0.05) in both types. Haemoglobin F and S were significantly higher (P less than 0.05) in SB0 than SB+ while haemoglobin A2 level was lower in SB0 than SB+. One SB0 case with exceptionally high HbF (32 per cent) with severe clinical course was found. In this case, the high HbF did not ameliorate the clinical severity. Heterogeneity in each type of sickle cell/beta-thalassemia is discussed. The differentiation of SB0 and sickle cell disease is best made on the basis of family study.
Bashir
N
N
Department of Biochemistry, School of Medicine, University of Science and Technology, Irbid, Jordan.
Barkawi
M
M
Sharif
L
L
D002648:Child; D002675:Child, Preschool; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis / Q000235:genetics; D015870:Gene Expression; D006443:Hemoglobin A2 / Q000032:analysis / Q000235:genetics; D006451:Hemoglobin, Sickle / Q000032:analysis / Q000235:genetics; D006801:Humans; D007597:Jordan / Q000453:epidemiology; D008297:Male; D012805:Sickle Cell Trait / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology; D013789:Thalassemia / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology
D006451:Hemoglobin, Sickle; D006443:Hemoglobin A2; D005319:Fetal Hemoglobin