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69 Cards in this Set
- Front
- Back
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Hemolytic disruption of the erythrocyte involves:
A.) an alteration in the erythrocyte membrane B.) a defect of the hemoglobin molecule C.) an antibody coating the erythrocyte D.) physical trauma |
A.) an alteration in the erythrocyte membrane
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Destruction of RBCs outside the circulatory blood
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Extravascular hemolysis
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Destruction of RBC inside the circulatory blood
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Intravascular hemolysis
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Which of the following tests is not useful in determining increased erythrocyte destruction?
A.) Reticulocyte count B.) Total leukocyte count C.) Serum haptoglobin D.) Unconjugated bilirubin |
B.) Total leukocyte count
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G6PD deficiency defect category
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erythrocytic enzyme defect
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Hereditary spherocytosis defect category
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structural membrane defect
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Thalassemia defect category
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Defect of the hemoglobin molecule
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Pyruvate kinase deficiency defect category
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erythrocytic enzyme defect
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Hereditary spherocytosis
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the most common prevalent hereditary hemolytic anemia among people of Northern European descent
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Hereditary elliptocytosis
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an overabundance of elliptic red cells
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Hereditary pyropoikilocytosis
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a subgroup of common hereditary elliptocytosis
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Hereditary stomatocytosis
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can be seen in the genetic hemoglobin defect, thalassemia
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Hereditary xerocytosis
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a permeability disorder
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Heinz bodies are associated with the congenital hemolytic anemia:
A.) G6PD deficiency B.) abetalipoproteinemia C.) hereditary spherocytosis D.) hemolytic anemias |
A.) G6PD deficiency
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A hemolytic crisis may be precipitated in 10% of American black males suffering from G6PD deficiency by:
A.) fava beans B.) primaquine C.) quinine D.) quinidine |
B.) primaquine
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What is the most common glycolytic enzyme deficiency associated with the aerobic pathway of erythrocyte metabolism?
A.) G6PD B.) pyruvate kinase C.) methemoglobin reductase deficiency D.) hexokinase deficiency |
A.) G6PD
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What is the most common glycolytic enzyme deficiency associated with the anerobic pathway of erythrocyte metabolism?
A.) G6PD B.) pyruvate kinase C.) methemoglobin reductase deficiency D.) hexokinase deficiency |
B.) pyruvate kinase
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What laboratory assay would specifically indicate a deficiency of G6PD enzyme?
A.) Heinz bodies on peripheral blood smears B.) Reticulocyte count C.) Hemoglobin and hematocrit D.) Osmotic fragility test |
A.) Heinz bodies on peripheral blood smears
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What enzyme deficiency causes methemoglobinemia?
A.) G6PD B.) pyruvate kinase C.) methemoglobin reductase deficiency deficiency D.) hexokinase |
C.) methemoglobin reductase deficiency
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Acquired hemolytic anemia can be caused by:
A.) chemicals or drugs B.) infectious organisms C.) antibody reactions D.) all of the above |
D.) all of the above
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The infectious microorganism directly associated with hemolytic uremic syndrome is:
A.) Pasteurella tularensis B.) E. coli O157-H7 C.) Staphylococcus aureus D.) Clostridia botulinum |
B.) E. coli O157-H7
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Warm-type autoimmune hemolytic anemia is associated with?
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Rh antibodies are the most frequent cause
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Cold-type autoimmune hemolytic anemia is associated with?
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IgM, usually anti-I
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Isoimmune hemolytic anemia
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Usually occurs in newborn infants
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The erythrocyte alteration characteristically associated with hemolytic anemias is:
A.) hypochromia B.) macrocytosis C.) spherocytosis D.) burr cells |
C.) spherocytosis
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What laboratory procedures would reflect a typical hemolytic anemia?
A.) increased osmotic fragility B.) increased total serum bilirubin C.) increased reticulocyte count, unless hematopoiesis is suppressed D.) all of the above |
D.) all of the above
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Which of the following is not associated with hemolytic anemia?
A.) decreased hemoglobin and packed cell volume B.) increased reticulocyte count C.) increased serum haptoglobin D.) decreased erythrocyte survival |
C.) increased serum haptoglobin...should be decreased serum haptoglobin
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Paroxysmal nocturnal hemoglobinuria exhibits sensitivity of one population of red blood cells to:
A.) warm antibodies B.) cold antibodies C.) complement D.) either A or B |
C.) complement
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Paroxysmal nocturnal hemoglobinuria episodes are usually associated with:
A.) cold temperatures B.) hot temperatures C.) sleep D.) certain foods or drugs |
C.) sleep
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The defect in paroxysmal nocturnal hemoglobinuria probably is a______associated defect of the red cell membrane.
A.) structural B.) hemoglobin C.) biochemical D.) both A and C |
D. both structural and biochemical
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Hemolytic anemia
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erythrocyte destruction is initiated by trapping of cells in sinuses of the spleen or liver and producing a decrease in the average life span of the RBC
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intrinsic hemolytic anemia
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inherited
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extrinsic hemolytic anemia
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acquired
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extravascular hemolysis
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site of destruction is outside the circulatory system like the liver or spleen
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inherited/instrinsic hemolytic anemia
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may affect:
- the basic membrane structure - RBC enzymes - the hemoglobin molecules within the red cells |
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Causes of membrane defects
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- genetic mutations of RBC cytoskeleton
- disorders of cation permeabiity - abnormal lipid bilayer or integral membrane protein |
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Examples of inherited hemolytic anemia (structural membrane defects)
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- hereditary spherocytosis
- hereditary elliptocytosis - hereditary pyropoikilocytosis - hereditary stomatocytosis - hereditary xerocytosis |
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hereditary spherocytosis
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- most common prevalent hereditary hemolytic anemia among people of Northern European descent
- defect in the interaction between the cell membrane skeleton and bilayer - extravascular hemolysis in the presence of the spleen |
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hereditary spherocytosis hemoglobin concentration
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normal or decreased
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HS MCV
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normal or slightly decreased
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HS MCHC
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increased
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HS osmotic fragility test
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positive
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hereditary elliptocytosis
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- caused by defects in horizontal interactions between components of the membrane skeleton
- shows little or no hemolysis |
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hereditary pyropoikilocytosis
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- a subset of HE
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hereditary pyropoikilocytosis MCV
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- decreased due to microspherocytes and fragments
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hereditary stomatocytosis
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- can be seen in the genetic hemoglobin defect, thalassemia, lead poisoning, alcoholic cirrhosis
- cation abnormality, water enters cell and overhydrates |
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hereditary stomatocytosis MCHC
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- usually decreased
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hereditary stomatocytosis MCV
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- may be increased
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hereditary stomatocytosis bilirubin
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- increased
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hereditary xerocytosis
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- permeability disorder
- the loss of K+ exceeds the Na+ influx, results in increase intracellular Na causing cell to dehydrate |
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Rh Null Disease
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- caused from the deletion of all Rh determinants
- permeable to K+ which leaks out of cell |
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Acanthocytosis
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- in abetalipoproteinemia and spur cell anemia
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erythrocytic enzyme deficiencies
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1.) Glucose-6-phosphate dehydrogenase
2.) Pyruvate kinase deficiency 3.) Methemoglobin reductase deficiency |
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Glucose-6-phosphate dehydrogenase deficiency
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- primaquine can induce a hemolysis in blacks
- chloramphenicol, quinine, quinidine, and fava beans can cause hemolysis in non-blacks - in pentose-phosphate pathway (aerobic-glycolytic pathway) - NADPH not produced, so oxidized glutathione accumulates |
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G6PD deficiency lab results
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- positive autohemolysis test
- Heinz bodies |
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Pyruvate kinase deficiency
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- in the Embden-Meyerhof pathway of anaerobic glycolysis
- loss of water and ATP causes cell shrinkage |
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pyruvate kinase deficiency
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- elevated 2,3DPG
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Methemoglobin reductase deficiency
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- increased methemoglobin
- heme is oxidized into ferric instead of ferrous form |
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Acquired hemolytic anemia
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- chemicals, drugs, venom, infectious microorganisms, immune mechanisms (Ig or complement), physical agents (burns), trauma (DIC) and microangiopathic hemolytic anemias
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Autoimmune hemolytic anemia
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- antibody against RBC
- due to warm-reactive or cold-reactive antibodies |
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Warm-type autoimmune hemolytic anemia
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- IgG coating of RBCs
- autoantibodies directed at Rh antigens - interaction between Fc portion of Ig and Fc receptor of splenic macrophages, therefore can't pass (extravascular) |
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Cold-type autoimmune hemolytic anemia
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- IgM coat RBCs
- if complement activated, then intravascular hemolysis |
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Isoimmune hemolytic anemia
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- in newborns
- transplacental passage maternal antibodies against antigens of baby's cells - ABO incompatibility |
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Unconjugated bilirubin
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when RBC destruction is increased and liver can't conjugate it
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Hemoglobinuria
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when RBC catabolism and free hemoglobin increases, haptoglobin is used up and can't stop it from going through the glomeruli
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Hemolytic anemia Hgb, Hct, and RBC
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decreased
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Reticulocyte count in hemolytic anemia
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increased
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serum haptoglobin in hemolytic anemia
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decreased
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Paroxysmal Nocturnal Hemoglobinuria (PNH)
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- chronic hemolytic anemia, thrombosis, and pancytopenia
- intermittent, sleep, blood in urine - intravascular hemolysis |
