Tags
#haematology #medicine
Question
What is the pathophysiology of Sickle-cell anaemia ?
Answer
- Production of abnormal globin chains.
- An amino acid substitution in the gene coding for the chain (Glu Val at position 6 ) results in the production of HbS rather than HbA.
- HbS polymerizes when deoxygenated, causing RBCs to deform, producing sickle cells, which are fragile and haemolyse, and also block small vessels
- HbA 2 and HbF are still produced.
Tags
#haematology #medicine
Question
What is the pathophysiology of Sickle-cell anaemia ?
Tags
#haematology #medicine
Question
What is the pathophysiology of Sickle-cell anaemia ?
Answer
- Production of abnormal globin chains.
- An amino acid substitution in the gene coding for the chain (Glu Val at position 6 ) results in the production of HbS rather than HbA.
- HbS polymerizes when deoxygenated, causing RBCs to deform, producing sickle cells, which are fragile and haemolyse, and also block small vessels
- HbA 2 and HbF are still produced.
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