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Sickle cell-hereditary persistence of fetal haemoglobin and its differentiation from other sickle cell syndromes
N Murray1, B E Serjeant, G R Serjeant
1Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston.
Some haematological indices were compared in 13 subjects with sickle cell-hereditary persistence of fetal haemoglobin (S-HPFH) and in 10 patients with homozygous sickle cell (SS) disease and four patients with sickle cell-beta zero thalassaemia, all with fetal haemoglobin (HbF) levels of 20% or above. Sickle cell-beta zero thalassaemia could be differentiated by a high HbA2 level. S-HPFH could be distinguished from SS disease with a high HbF level by red cell count, HbF level, reticulocyte count, total haemoglobin and total bilirubin level in decreasing power of discrimination.
Some haematological indices were compared in 13 subjects with sickle cell-hereditary persistence of fetal haemoglobin (S-HPFH) and in 10 patients with homozygous sickle cell (SS) disease and four patients with sickle cell-beta zero thalassaemia, all with fetal haemoglobin (HbF) levels of 20% or above. Sickle cell-beta zero thalassaemia could be differentiated by a high HbA2 level. S-HPFH could be distinguished from SS disease with a high HbF level by red cell count, HbF level, reticulocyte count, total haemoglobin and total bilirubin level in decreasing power of discrimination.
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