- Autosomal recessive
- Homozygous inheritance of HbS
- Single amino acid substitution of valine for glutamic acid in 6th position of B-chain of HbA.
- When oxygen tension low (deoxygenated);
- HbS crystallises
- cell becomes crescent
- trapped
- microinfarction.
- Children get splenomegaly
- Acute manifestations mainly relate to:
- infections (due to functional asplenia)
- Aneamia
- Vaso-occultion
- Treatment
- Hydroxyurea
- increases production of fetal hemoglobin that then interferes with the hemoglobin polymerisation as well as by reducing white blood cells that contribute to the general inflammatory state in sickle cell patients.
- Transfusions
- Splenectomy vaccinations
- Indications for surgery include hypersplenism, Acute splenic sequestration crisis which lead to requirements for blood transfusion, development of splenic abscess, splenic infarction, pain.