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27 Cards in this Set
- Front
- Back
During the first 2 months of development in the ___, ____ and ____ Hb diminish, while the synthesis of ____ and ____ HB increase, and ____ Hb begins.
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yolk sac, epsilon, zeta, gamma A & G, alpha, beta
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From 2 months to birth, in the ____ and ____, ___ Hb is produced at adult levels, while _____ and ____ Hb increase and decrease at 6 months of development, respectively.
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liver, spleen, alpha, beta, gamma
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At birth, HbF is present composed of ____, and HbA is present composed of ___, and HbA2 begins to form and is composed of _____.
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2 alpha and 2 gamma subunits, 2 alpha and 2 beta, 2 alpha and 2 delta
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Hb subunits are made from genes on chromosomes ___ and ___.
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16, 11
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Chromosome 16 contains the zeta subunit, pseudo zeta and alpha subunits, and ___.
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alpha 2 and alpha 1 subunits
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Chromosome 16 contains the pseudo beta, epsilon, gamma A & G, pseudo beta, ____ and ___ subunits
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delta, beta
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Underproduction of alpha chains is ____ thalasemia, while underproduction of beta chains is ____ thalasemia.
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alpha, beta thalassemia
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____ is heme oxidation to its ferric state that is a non function Hb.
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Methemoglobinemia
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Methemoglobinemia is caused by poisoning with ____ agents, overproduction of ___ by enzymes, failure of methemoglobin ____, and congenital replacement of His by ___.
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oxidizing, oxidants, reductase, Tyr
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Abnormal Hb structre are usually ____ mutations.
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point
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Thalassemia ___ is a homozygous disorder, while thalassemia ____ is a heterozygous disorder.
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major, minor
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A sign of thalassmeia with low Hb/RBC and small sized RBC
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Hypochromic microcytic anemia
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In thalassemias, abnormal cells with dark spots in the center, formed from the abnormal aggregates of Hb subunits.
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target cells
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___ are RBC of unequal sizes and ____ are variable shapes, which are symptoms of thalassemia.
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angiocytosis, poikilocytosis
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___ thalassemia is characterized as having no beta chains, while ___ i having few beta chains.
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beta 0, beta+
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___ thalassemia major is characterized with severe anemia but not at birth.
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beta
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____ thalassemia minor will see increased HbA2 and sometimes increased HbF.
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beta
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Abortive erythropoiesis, increase in bone marrow, hepatosplenomegaly, and pigment stones are do to ____.
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increased phagocytosis of RBCs in spleen and liver
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Treatment of beta thalassemia includes blood transfusions which increases already increased ____ absorption, so deferrioxamine must be administered.
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iron
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Azacytidine reactivate ____ subunits, but is not well tolerated by the patient.
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gamma
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HbS is a mutation in the ___ subunit that can form ____ in its deoxygenated form.
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beta, precipitates
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HbS is a point mutation in the beta subunit on chromosome 11 that replaces a hydrophilic Glu with a hydrophobic ____.
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Val
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Out of alpha thalassemia, beta thalassemia, and sickle cell anemia, only ___ shows signs at birth.
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Alpha
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In hydrops fetalis ___ loci are deleted resulting in ___Hb, composed of tetrameric γ chains.
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4, barts
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In alpha thalassemia the deletion of ___ loci results in moderately severe anemia and HB __ disease.
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3, H (tetrameric β chains)
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In beta thalassemia patients are treated with folic acid to stimulate ___, or receive a _____, to increase the time RBCs spend in the blood.
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erythropoesis, slenectomy
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In order for sickling to occur the patient must be ____ affected, if they are ____ they have improved malaria resistance.
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homozygous, heterozygous
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