Sickle cell-hereditary persistence of fetal haemoglobin and its differentiation from other sickle cell syndromes.

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.