All the following are true about sickle cell anemia except
Ref Harrison 19 th ed pg 634 The sickle cell syndromes are caused by a mutation in the b-globin gene that changes the sixth amino acid from glutamic acid to valine. HbS (a2 b2 6 Glu-Val) polymerizes reversibly when deoxygenated to form a gelatinous network of fibrous polymers that stiffen the RBC membrane, increase viscosity, and cause dehydration due to potas- sium leakage and calcium influx.
Mutation in alpha chain
Symptoms ameliorated by Hbf
Venoocclusive cries is cause of morbidity
Bone pain is presenting feature
Haematology