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52 Cards in this Set
- Front
- Back
Hemoglobin; FUNCTION
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Transport oxygen from the lungs to the tissues and CO2 from the tissues back to the lungs
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The Bohr Effect
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Decreased 02 affinity in an acid environment, increased affinity in basic environment
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Tissues have ___ ph compared to lungs
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lower
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In lungs O2 taken up by Hb as environment is more ___
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alkaline
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beta globin gene cluster chromosome -
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11
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alpha globin gene cluster chromosome -
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16
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The combination of two alpha chains and two gamma chains form "fetal" hemoglobin, termed "
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hemoglobin F
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The combination of two alpha chains and two beta chains form "adult" hemoglobin, also called
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hemoglobin A
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The expression of the alpha and non-alpha genes is closely balanced by an unknown mechanism. Balanced gene expression is required for normal red cell function. Disruption of the balance produces a disorder called .
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thalassemia
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Term used to describe cell death
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SENESCENCE
In the steady state the rate of red cell production per day is equivalent to the rate of red cell senescence |
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Complaints
Fatigue Shortness of breath Chest pain Forgetfulness Apathy Physical Exam Pale Ill appearing Icteric – yellow sclerae Tachycardic (acute) Pulmonary crackles (heart failure?) Splenomegaly Stomatitis, chelitis Nail changes |
The Anemic patient
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A measure of the average size of the red blood cells
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MCV
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Measures the variation in red blood cell size present in a sample of blood
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Red cell distribution width
When the cells are uniform in size the RDW is normal When the cells exhibit a lot of variation in their size the RDW is high |
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means that the red blood cells have less color than normal when examined under a microscope or to be more scientific..
Lack of complete saturation of the erythrocyte stroma with hemoglobin, as judged by pallor of the unstained or stained erythrocytes when examined microscopically. |
Hypochromia
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The average normal North American diet contains ___ mg of iron. Most is present in meat and green vegetables.
Approximately 1.0 mg is absorbed each day and about an equal amount is lost in the feces and sweat. |
15-20
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a protein present in the blood essential in the transport of iron from the intestine into the bloodstream, making it available to the normoblasts in the bone marrow.
When not bound to iron, it is known as "_____" |
apo-transferrin
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a protein that stores iron and releases it in a controlled fashion
a "buffer" against iron deficiency when it is combined with iron is called |
Ferritin
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What Type of Surgery Would Predispose A Patient To Iron Deficiency?
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Bariatric surgeryDuodenum surgery
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What Medications Would Impair Iron Absorption?
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Proton pump inhibitorsAntacids
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Iron is stored as either
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ferritin or hemosiderin
Ferritin is water soluble and found mainly in the bone marrow and liver. It is preferentially used before hemosiderin |
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Most common anemia in the world
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Iron Deficiency Anemia
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Autosomal recessive
Most common genetic disorders in Northern Europeans 10% of the population are heterozygotes 0.5% homozygous (major problem) |
Hereditary Hemochromatosis
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Weakness,fatigue and chronic abdominal pain
Abnormal liver function with eventual cirrhosis Congestive heart failure Arthritis (metacarpal joints) Diabetes Hyperpigmentation “the bronzed diabetic” |
Hereditary Hemochromatosis
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___ is a must for absorption, as it releases B12 from protein *this is highly clinically significant
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Acid secretion
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recognizes UTP as a mismatch and excises it from DNA strand
No TTP available to replace UTP DNA becomes fragmented leading to arrest of DNA synthesis at various stages |
Uracil glycosylase
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why is folate and B12 important
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bc it is important in oncology - without them, we will have fragmented DNA!
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Defined as defect in DNA synthesis with a lesser effect on RNA and protein synthesis
Leads to unbalanced cell growth and impaired cell division |
MEGALOBLASTOSIS
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Bone marrow is hypercellular with a erythroid predominance
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MEGALOBLASTOSIS
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Pathognomonic of
megaloblastic anemias |
Hypersegmented Neutrophils
Normal neutrophils contain three or four nuclear lobes, Hypersegmented neutrophils contain five or more |
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Anemia
Pallor Lemon yellow tint to skin Glossitis (SHINY PAINFUL TONGUE) Neurologic abnormalities- loss of position sense, gait disturbances, weakness, spasticity |
MEGALOBLASTOSIS
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Lesions can be seen throughout the length of the spinal cord and white matter of the brain
Incoordination Difficulty walking Sensory disturbances Diminished position and vibration sense Weakness Spasticity |
B12 Deficiency
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Making the Diagnosis of B12 and or Folate Deficiency
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Clinical suspicion
Measure serum B12 and folate levels prior to feeding patient One meal can normalize serum folate levels in a deficient patient Red blood cell folate will be low in folate deficiency Red blood cell folate will be normal in B12 |
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T cell infiltrate gastric mucosa leads to Atrophy of gastric mucosa leading to absence of intrinsic factor and HCL acid secretion
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Pernicious Anemia
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The Schilling’s Test
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1.0 mcg of radioactively labeled B12 orally and 1000 mcg B12 IM
Determine radioactivity excretion in urine If low, oral B12 not properly absorbed Give Intrinsic factor, if corrects then PA Part 2 give 7-10 d antibiotics, if corrects then bacterial overgrowth Part 3 give pancreatic enzymes, if corrects then pancreatic dysfunction (insufficiency) |
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Splenectomy as abnormal cells are destroyed in the spleen
Relapses due to accessory spleen Life threatening infections can occur in splenectomized patients Vaccines crucial |
Treatment of HS
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Most common type of AIHA
Autoantibodies attach to the RBC surface Can occur w/o underlying cause but may also be first presentation of lymphoma, lupus and CLL, also infections such as TB and CMV, chronic inflammatory disorders such as Crohn’s disease |
IgG
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Used to determine if antibodies are present
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The Coombs test
Direct Coombs measures antibodies on the RBC Indirect measures antibodies in the serum (used for blood typing for transfusion) |
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tx of IgG AIHA
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Corticosteroids
Gamma globulin Splenectomy Immunosuppressive agents Transfusions may be difficult because of presence of antibodies and cross reactivity with donor red blood cells |
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a rare disorder of the coagulation system, causing extensive microscopic blood clots to form in the capillaries
Red blood cells passing the microscopic clots are subjected to shear stress which damages their membranes, leading to intravascular hemolysis. |
TTP
Thrombotic thrombocytopenic purpura (TTP |
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Classically, the ("pentad") is indicative of TTP
**** know the pentad! |
Fluctuating neurological symptoms, such as bizarre behavior, altered mental status, stroke or headaches
Kidney failure Fever Thrombocytopenia, leading to bruising or purpura Hemolytic anemia |
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symptoms:
Fluctuating neurological symptoms, such as bizarre behavior, altered mental status, stroke or headaches Kidney failure Fever Thrombocytopenia, leading to bruising or purpura Hemolytic anemia |
TTP!!! ****
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tx of TTP
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Early and aggressive plasmapheresis
Steroids Rituxan |
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prognosis of TTP
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The mortality rate is approximately 95% for untreated cases ****************************************
(80-90%) for patients with idiopathic TTP diagnosed and treated early Secondary TTP still has a dismal prognosis, with mortality rates despite treatment being reported as 59% to 100%. |
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Mutation of B (beta) globin gene
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Sickle Cell Anemia
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Two major physiologic processes of sickle cell anemia
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Two major physiologic processes,
shortened red cell survival and vasoocclusion, account for most of the clinical manifestations of sickle cell disease. |
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Results from rigid, sickled red cells leading to the obstruction of blood vessels .
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Vasoocclusive
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in a hemolytic crisis why does the pt look yellow?
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The resulting increase in jaundice is associated with a falling hemoglobin level and an elevated reticulocyte count.
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Doesn't cure sickle cell disease, but it can reduce the number of painful events and other complications and may prolong life
Increases fetal hemoglobin(HbF) production and raises the total hemoglobin concentration |
Hydroxyurea
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stem cell disorder, characterized by increased progenitor cell sensitivity to growth-promoting cytokines and is strongly associated with an activating somatic mutation in the JAK2 tyrosine kinase.
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Polycythemia Vera
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Thrombosis and Bleeding are the major causes of morbidity and mortality from what
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polycythemia vera
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talk about creation of RBCs
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kidneys sense how much oxygen is in the blood... produces erythropoetin... sends it to BM which stimulated production of RBCs
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if more iron is required what happens to the amount of apoferritin manufactured in eat mucosal cell
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decreased apoferritein manufactured
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