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36 Cards in this Set
- Front
- Back
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what part of the world is HgE found?
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southeast Asia
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when is zeta hemoglobin present?
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embryonic development
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when is epsilon hemoglobin seen?
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embryonic development
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when is gamma hemoglobin seen?
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in fetal hemoglobin
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what type of hemoglobin does
zeta/epsilon alpha/epsilon zeta/gamma make? |
Hb Gower 1
Hb Gower 2 Hb Portland |
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What type of hemoglobin does
alpha/beta alpha/gamma alpha/delta make? |
Hb A
Hb F Hb A2 |
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What hemoglobin does
beta tetramer gamma tetramer make? |
Hb H
Hb Bart's |
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When can you detect Hb Bart's?
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at birth
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what is hemoglobin constant spring?
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alpha globin variant in persons of asian ancestry with a mutation at the termination codon?
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what is hemoglobin H disease?
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3 gene deletion of alpha globin
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What is the MCV of a child with Hb H disease at birth?
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MCV < 100 ie microcytosis
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What hemoglobins are seen after infancy in Hb H disease?
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Hb H (beta tetramer)
Hb A (alpha/beta) Hb Bart's (gamma tetramer) |
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Are Heinz bodies present in HB H disease?
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Yes b/c there are unstable hemoglobins
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Iron absorption in Hb H disease?
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increased.
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role of transfusions in Hb H disease?
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+/- dependent
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Electropheresis findings in beta thalassemia trait?
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Hb A
elevated Hb A2 (alpha/delta) > 3.5% elevated Hb F (alpha/gamma) |
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what are the electropheresis findings in beta thalassemia major?
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at birth only Hb F (alpha/gamma)
later Hb F and Hb A2(alpha/delta) |
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what is the goal in hypertransfusing beta thalassemia major patients
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turn off erythropoeisis
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is hemoglobin E a qualitative or quantitiative defect?
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quantitative: behaves like thalassemia
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morphologic abnormality in Hb C disease?
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target cells
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why is parvovirus particulary problematic in RBC disorders?
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there is a specific antigen (the P antigen) on the RBC precursor membrane that serves as receptor for parvovirus B19
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how does erythrocyte porphyrin level differentiate iron deficiency from thal trait (alpha or beta)
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level is elevated in iron deficiency but normal in thal trait.
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how many milligrams of iron are usually contained in a unit of transfused PRBCs?
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200mg
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in sickle cell, in the deoxygenated state what interaction is mutated
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hemoglobin tetramer to hemoglobin tetramer
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what is hgb H- constant spring
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a form of alpha thal intermedia that is more severe than classical Hgb H disease
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what is the most common hgb mutation world wide
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Hgb E
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what is the defect in Hgb E
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both qualitative and quantitative
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what do HPFH and HgB lepore have in common
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gamma chain synthesis compensates in both for defective delta and beta synthesis
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what is the difference in oxygen affinity between Hgb Kansas and Hgb syracuse
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kansas - decreased oxygen affinity
syracuse - increased oxygen affinity |
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In what geographcial locations are hgb D and hgb O found
clinical picture |
D- 'punjab' india, pakistan, iran
0 - 'arab' africa, middle east both produce sicle cell disease |
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what does
erythrocytosis Hgb F anemia cyanosis do to the oxy hemoglobin dissociation curve? |
erythrocytosis - high o2 affinity (curve to the left)
Hgb F - high affinity anemia - low affinity cyanosis - low affinity |
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what explains the quantitative reduction in the synthesis of HgB E
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abnormal cryptic splice site
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what is the most common hemoglobinopathy in the world
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Hgb E
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What type of mutational event most commonly causes the beta thalassemias
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point mutations
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what type of mutational event causes the alpha thalassemias
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deletion of the entire alpha globin genes
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what type of thalassemia spontaneously resolves in infancy, what are the findings in infancy?
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gamma thalassemia
in infancy: microcytic, hemolytic anemia |
