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Hgb F globulin chains
A and gamma
Hgb A2 globulin chains
A and S
Hgb A globulin chains
A and B
How does Hgb F affect oxygen binding
much higher binding affinity
Hgb S globulin chain
B chain
What is SCD
when a patient has a sickle cell gene and B thalassemia gene
Where on the B chain is the sickle cell mutation
position 6, glutamic acid replaced by valine
What does the change in RBC charge in sickle cell do to the RBC
changes how RBC interact (motility)
When do RBCs have the sickle cell RBC shape
when deoxygenated (<85% homo)
Reversible sickle cell
RBCs change shape in response to O2
Irreversible sickle cell
RBCs sickle regardless of O2
What causes the anemia in sickle cell
extravascular hemolysis (chronic)
BM in sickle cell
hyperplastic
Most common symptom of sickle cell in the ER
vaso-occlusive crisis: RBC aggregate in distant vasculature
Why do megaloblastic episodes occur in sickle cell
folate depletion due to attempt of compensation
Retinal ischemia
clotting in small eye capillaries
Avascular necrosis
impaired blood supply to head of femur/ humerus
Sickle cell anemia type
normocytic, normochromic
Retic count in sickle cell
10-25% marked polychromatic
SCD screen
presence of tactics (crystals)
SCD confirmatory test
hgb electrophoresis
What can cause a false positive SCD screen
hyperlipidemia
Alkaline electrophoresis does what
measure hgb A and S
Acidic electrophoresis does what
separates S from D and G
Best method of SCD confirmation
Capillary electrophoresis
What pathogen does SCD protect the patient from
Malaria (P. falciparum)
RBC morphology in Hgb C
crystals (gold bar)
Hgb C B chain change
lysine replaces glucose
What other anemia is needed to be differentiated from Hgb E
IDA
Hgb SC RBC morphology
dark red sharp RBC
Hgb M
methemoglobin (chocolate blood no O2)
Unstable hgb disease RBC morphology
Heinz bodies
