Sickle cell anaemia in Jordan and its clinical patterns.
Homozygous sickle cell anaemia (SCA) is an inherited red blood cell disorder in which haemoglobin A is replaced by haemoglobin S. The disease exhibits a wide spectrum of clinical behaviour which has been well described in neighbouring countries. In Jordan, however, the disease has never been characterized. We used reviews of patients' notes and clinic interviews at Princess Badi'a Teaching Hospital, Irbid, Jordan to describe the clinical presentation of the disease in our area. The total number of patients was 41 (28 boys and 13 girls) and the median age was 9 years (range 1.5-21). The median age at presentation was 2.5 years (range 0.5-11). The commonest presenting feature was pallor (62%). An unusual feature of the disease in this series is the presence of a palpable spleen in 44% of patients older than 8 years. Associated G6PD deficiency was present in 29% of boys. Different clinical patterns of SCA were observed. Haemoglobin F is unlikely to be an explanation for this variability since it has not been shown to correlate with a number of severity indices of the disease such as the frequency of blood transfusions, hospitalizations and painful crises. Although the disease haplotype is not known in Jordan, our geographical location between Asia and Africa may suggest the presence of more than one haplotype and consequently different clinical patterns.
Department of Paediatrics, Jordan University of Science and Technology, Irbid, Jordan.
M
M
al-Sheyyab
H
H
Rimawi
M
M
Izzat
A
A
Batieha
N
N
el Bashir
N
N
Almasri
A S
AS
Daoud
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications; D006239:Haplotypes; D006760:Hospitalization; D006801:Humans; D007223:Infant; D007597:Jordan; D008297:Male; D012720:Severity of Illness Index; D013163:Splenomegaly / Q000209:etiology
D005319:Fetal Hemoglobin