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36 Cards in this Set

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