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Hematology
Hematology NYCOM MS2
35
Medical
Not Applicable
11/20/2003

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Cards

Term
Name the earliest cell destined to become an RBC
Definition
BFU-E
Term
What is the first cell to have receptors for erythropoetin?
Definition
CFU-E
Term
List the erythroid developmental line
Definition
  • Multipotent stem cell
  • BFU-E
  • CFU-E
  • Proerythroblasts 4 types below
    1. basophilic erythoroblast
    2. polychromatophilic
    3. orthochromatophilic
    4. reticulocytes
  • Red cells
  • Term
    What is erythropoetin?
    Definition
    an obligatory growth factor for erythroid development
    Term
    Where is erythropoetin produced?
    Definition
    in the peritubular cells of the kidney
    Term
    What is the trigger for the synthesis of erythropoetin?
    Definition
    a heme containing protein within erythropoetin that senses oxygen need
    Term
    What stimulates the production of RBC's?
    Definition
    Erythropoetin transpoted through the blood to bone marrow where it binds to receptors on the CFU-E
    Term
    What happens to RBC production in renal failure?
    Definition
    it will decrease b/c the growth factor erythropoetin is made in the kidney, this is known as the anemia of kidney failure
    Term
    What happens to RBC production during hypoxia?
    Definition
    It will increase b/c low oxygen tension is the trigger for the synthesis of erythropoetin by the peritubular cells of the kidney
    Term
    What is the life span of an RBC?
    Definition
    120 days
    Term
    How many times will a RBC circulate through the heart in its lifetime?
    Definition
    170,000
    Term
    More than 95% of an RBCs protein is this?
    Definition
    hemoglobin
    Term
    What is the shape of an RBC?
    Definition
    a biconcave disc
    Term
    why is an RBC's shape important?
    Definition
    it facilitates gas transfer
    Term
    What important property of the RBC does the cytoskeleton allow for?
    Definition
    deformability
    Term
    Abnormalities of the cytoskeleton lead to what in RBC's?
    Definition
    pathological shapes such as elliptocytes and spherocytes
    Term
    What is the RBC cytoskeleton composed of?
    Definition
    1. glycolipids
    2. phospholipids
    3. integral proteins
    4. transmembrane proteins
    Term
    what are the 4 most well studied cytoskeleton proteins?
    Definition
    1. ankyrin
    2. spectrin
    3. band protein 4.1
    4. band 3 protein>
    Term
    What happens to RBC's when the cytoskeleton is abnormal?
    Definition
    abnormalities within cytoskeleton proteins lead to disorders where the RBC can't deform in the capillary beds and will die
    Term
    What is a reticulocyte?
    Definition
    a newly formed erythrocyte
    Term
    what differentiates a reticulocyte from a RBC?
    Definition
    reticulocytes still contain some RNA that remains for 1-2 days after the cell enters circulation, one that goes away it is a mature erythrocyte
    Term
    What is the normal reticulocyte count and how do we determine it?
    Definition
    1% of erythrocytes in circulation and we stain with supravital stain to obtain the reticulocyte count
    Term
    Under what conditions would you see an increaded reticulocyte count?
    Definition
    anemia, where the response is to increase RBC production
    Term
    From what causes of anemia would you see a low reticulocyte count?
    Definition
    with bone marrow problems and iron deficiency
    Term
    What is the purpose of hemoglobin?
    Definition
    to reversibly bind oxygen
    Term
    How much more oxygen can hemoglobin+plasma carry vs. plasma alone?
    Definition
    100x's more
    Term
    What is a heme?
    Definition
    it is a complex of iron and protoporphyrin
    Term
    What is the structure of a hemoglobin molecule?
    Definition
    a tetramer consisting of 2 pairs (4 total) similar polypeptide chains and to each of the 4 chains is attached a heme (iron and protoporphyrin)
    Term
    Where is the hemoglobin alpha gene locus and what two proteins does it produce?
    Definition
    chromosome 16 and globins alpha and sigma(only during first 8 weeks of fetal development and will mature into alpha)
    Term
    Where is the hemoglobin beta gene locus and what protiens does it produce?
    Definition
    chromosome 11 and produces fetal hemoglobin and adult hemoglobin (delta & beta)
    Term
    RBC's containing which hemoglobin have the highest affinity for O2?
    Definition
    fetal hemoglobin
    Term
    what are the normal hemoglobin components in adults?
    Definition
    Hgb A1 (a2b2), A2(a2d2), and up to 2% F (a2f2)
    Term
    What happens to the hemoglobin in pediatric popultion with increasing age?
    Definition
    HbF can be as high as 80% in a newborn and will decrease to look more like the normal adult count
    Term
    What two types of hemoglobin should never be seen in panel?
    Definition
    2 abnormals= HgS (sickle cell) and HgC (abnormal)
    Term
    What are the requirements for hemoglobin to function as an exchange medium between O2 and CO2?
    Definition
    1. capable of transporting large amounts of O2
    2. Highly soluble
    3. Take up and release O2 at appropriate pressures
    4. be a good buffer
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