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15 Cards in this Set

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Name the 3 major Hemoglobins that are present in mature red blood cells
Hemoglobin A(98%), A2, and F
How do the various Hemoglobins differ and how are they similar?
They all share globin chain alpha but differ in the second globin chain.
What feature allows Hb F to cross the placenta?
It's affinity for oxygen.
When does rbc production begin in the embryo?
By day 19 in the yolk sac.
What are the forms of embryonic Hb? & What how do they fxn after birth?
Gower 1, Gower 2, and Portland. These Hb's are of no clinical importance after birth.
These are defined as mutations that affect the production of normal Hb chains
Thalessemia syndromes
What are 4 major genetic abnormalities that can occur in globin synthesis?
1. Decreased solubility
2. Oxygen Transport (w/ high/low O2 affinity)
3. Oxygen reduction status
4. Monomeric state
Why do most of the hemoglobinopathies affect the beta chain?
Because there are 4 copies of the alpha chain.
Name the 4 types of hemoglobinopathies studied?
1) sickle cell syndromes
2) unstable Hb's
3) Hb's w/ altered O2 affinity
4) Hb M
What are some promoters of rbc cell sickling?
hypoxia, low pH, 2,3DPG, reduced rbc H2O, and fever
Name two tests for sickle cell syndrome?
1) sickle prep test w/ dithionate or metabisulfate

2) Hb electrophoresis
This is the dominant cause of morbidity and mortality in HbSS (sickle cell anemia)
vasocclusive crisis
What is splenic sequestration crisis?
the spleen enlarges in kids and Hb drops; the child bleeds into the spleen
Aplastic crisis
Is due to Parvovirus B19 or secondary folic acid deficiency; occurs when rbc production turns off
Name two tests for sickle cell syndrome?
1) sickle prep test w/ dithionate or metabisulfate

2) Hb electrophoresis