13 Sickle Cell
- 2 genes, 1 from each parent
- A to T leads to aa substition
- dehydration/acidosis: acute exacerbation
- polymerize and make RBC form crescents
- decrease ESR instead of up
Symptoms
- hemolytic anemia similar to beta thalassemia major
- vaso occlusion: more dangerous and unique symptoms
Hemolysis
- sometimes simply rupture in vasculature
Vasoocclusion
- dehydration lead to sickling
- can become dependent on narcotics
- have spleen but not working
- can die from infection
- salmonella encapsulated, staph not
- The spleen may demonstrate brownish discoloration (hemosiderosis) due to extensive ingestion of sickled RBCs by splenic macrophages (extravascular hemolysis).
- fish bone: Osteomyelitis
- sickle: In Sickle Cell disease
- sickle: Increased risk of infection in sickle cell or asplenic patients
- pooling of RBC in spleen from obstruction
- children: spleen not yet fibrosis
- treated with antibiotics because looks just like pneumonia. Don't know if microbe present or not
- high concentration in medulla abolished
Treatment
Trait
Diagnosis
Malaria
- most severe form
- just as likely to be infected, less likely to die
Thalassemia
- less beta production depending on how severe infection is
HbC
- lysine more polar than valine: not as severe of a problem
- lysing more positive than glutamate, less travel than HbS and Hb normal on electrophoresis
- very rare to be homozygous
Hb SC
- heterozygous: usually mild anemia without sickling
- except HbS plus HbC
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