vaso occlusion

Sickle Cell Disease

Epidemiology

 

Black population

- heterozygote protective from malaria

- AD

 

Pathogenesis

 

Abnormal peptide substitution in ß chain of Hb

- HbS

 

Homozygous 1%

- HbSS

- sickling occurs with relative hypoxia

- trapped in blood vessels

- necrosis & pain

 

Heterozygous 8%

- sickle cell trait

- Hb SA

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