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27 Cards in this Set

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