A benign sickle-cell disease in a Saudi subject with beta zero-thalassemia and glucose-6-phosphate dehydrogenase deficiency.
Sickle-cell disease with raised fetal hemoglobin is found relatively frequently in the eastern part of the Arabian Peninsula. In contrast to the severe and sometimes life-threatening complications of sickle-cell disease in the black population, Saudi Arabs homozygotes for HbS gene exhibit a mild course for this disease. Here we present a Saudi sickle-cell patient with an unusually low fetal hemoglobin level. Moreover, this individual has beta 0-thalassemia and a deficiency in the enzyme glucose-6-phosphate dehydrogenase. Clinical and hematological examinations revealed a remarkably benign condition. This observation is potentially important since most of the mild clinical symptoms of sickle-cell disease have been attributed to high fetal hemoglobin. Clearly in this case, other factors are operating and may be also operating in those patients with high fetal hemoglobin.
Alayash
A I
AI
Duke University Marine Laboratory, Pivers Island, Beaufort, N.C.
Bonaventura
J
J
al-Quorain
A
A
D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D005319:Fetal Hemoglobin / Q000032:analysis; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications*; D006403:Hematologic Tests; D006801:Humans; D008297:Male; D012529:Saudi Arabia; D013789:Thalassemia / Q000097:blood / Q000150:complications*
D005319:Fetal Hemoglobin