Role of Coagulation in Endothelial Cells
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Questions and Answers

What is the primary function of kallikrein in the coagulation pathway?

  • Acceleration of factor XII activation (correct)
  • Stabilization of fibrin clots
  • Inhibition of thrombin formation
  • Regulation of platelet aggregation

    • Which of the following factors is responsible for the conversion of plasminogen to plasmin?

  • Factor XIIa
  • Fibrin stabilizing factor
  • Tissue factor
  • Kallikrein and activated factor XII complex (correct)

    • What is the function of high-molecular-weight kininogen (HMWK) in the coagulation pathway?

  • Activation of proaccelerin
  • Regulation of calcium levels
  • Mediation of inflammatory responses (correct)
  • Stabilization of fibrin clots

    • Which factor is responsible for the formation of a stable fibrin clot?

    <p>Fibrin stabilizing factor (XIII)</p> Signup and view all the answers

    What is the function of prekallikrein in the coagulation pathway?

    <p>Acceleration of factor XII activation</p> Signup and view all the answers

    What is the primary function of antithrombin III in the coagulation cascade?

    <p>To inhibit thrombin and other clotting factors</p> Signup and view all the answers

    Which of the following is NOT a naturally occurring inhibitor of fibrinolysis?

    <p>Protein C</p> Signup and view all the answers

    What is the primary mechanism of action of alpha-2-macroglobulin in inhibiting thrombin?

    <p>It binds to thrombin, slowing its activity</p> Signup and view all the answers

    Which of the following is an example of an intrinsic plasminogen activator?

    <p>Factor XIIa</p> Signup and view all the answers

    What is the primary function of C1 inactivator in the coagulation cascade?

    <p>To inhibit the contact system</p> Signup and view all the answers

    Which of the following inhibitors of the fibrinolytic system binds to both tissue plasminogen activator and urokinase in a 1:1 complex?

    <p>Plasminogen activator inhibitor-1</p> Signup and view all the answers

    What is the primary function of the activated protein C complexed with protein S in the coagulation pathway?

    <p>Destroying factors Va and VIIIa</p> Signup and view all the answers

    Which of the following substances is released from intact endothelial cells and has an anticoagulant effect?

    <p>Prostacyclin</p> Signup and view all the answers

    What is the consequence of a congenital deficiency of plasminogen activator inhibitor-1 (PAI-1)?

    <p>A bleeding disorder due to unopposed fibrinolysis</p> Signup and view all the answers

    Which of the following inhibitors is a potent inhibitor of factor Xla?

    <p>Alpha-1 antitrypsin</p> Signup and view all the answers

    Which factor is responsible for initiating the intrinsic coagulation pathway?

    <p>Factor XIIa</p> Signup and view all the answers

    What is the primary function of the von Willebrand portion of Factor VIII?

    <p>Platelet function</p> Signup and view all the answers

    Which of the following factors are substrates for the fibrinolytic enzyme plasmin?

    <p>Factors I, V, VIII, and XIII</p> Signup and view all the answers

    What is the primary function of the contact group?

    <p>Kinin formation and activation of fibrinolysis</p> Signup and view all the answers

    What is a common consequence of vitamin K deficiency or administration of antibiotics that sterilize the intestinal tract?

    <p>Impaired coagulation</p> Signup and view all the answers

    Which factor is activated by the factor XIa, HMWK, and calcium ions?

    <p>Factor IX</p> Signup and view all the answers

    What is the primary role of the tissue factor:factor VII complex in the coagulation cascade?

    <p>Conversion of factor X to Xa</p> Signup and view all the answers

    What is the primary source of plasminogen in the fibrinolytic system?

    <p>Liver</p> Signup and view all the answers

    Which of the following factors is responsible for the breakdown of coagulation factors?

    <p>Plasmin</p> Signup and view all the answers

    Which of the following substances is NOT an anticoagulant released from intact endothelial cells?

    <p>Fibrinogen</p> Signup and view all the answers

    What is the primary function of anti-plasmins in the fibrinolytic system?

    <p>Inhibition of plasmin activity</p> Signup and view all the answers

    What is the primary function of tissue plasminogen activator (TPA) in the regulation of hemostasis?

    <p>To convert plasminogen to plasmin, promoting fibrinolysis</p> Signup and view all the answers

    What is the consequence of increased plasmin levels in liver disease or certain cancers?

    <p>Excessive coagulation factor destruction</p> Signup and view all the answers

    Which of the following coagulation factors is associated with the surface receptor for thrombin and enhances anticoagulant and fibrinolytic actions?

    <p>Thrombomodulin</p> Signup and view all the answers

    Which of the following factors is involved in the activation of factor XI to XIa?

    <p>Factor XIIa</p> Signup and view all the answers

    What is the primary function of the fibrinolytic system?

    <p>Breakdown of fibrin clots</p> Signup and view all the answers

    What is the primary role of heparan sulfate in the regulation of hemostasis?

    <p>To weakly enhance the activity of antithrombin-III</p> Signup and view all the answers

    Which of the following factors is involved in the fibrinolytic system and stabilizes fibrin clots?

    <p>Fibrin stabilizing factor (Laki-Lorand Factor)</p> Signup and view all the answers

    Which of the following is a component of the intrinsic pathway of coagulation?

    <p>Factor XI</p> Signup and view all the answers

    What is the primary function of calcium ions in the coagulation cascade?

    <p>Required for the activity of many coagulation factors</p> Signup and view all the answers

    What is the primary function of non-enzymatic cofactors in the coagulation pathway?

    <p>To alter zymogen conformation to permit more efficient cleavage by serine proteases</p> Signup and view all the answers

    Which of the following factors is NOT a component of the extrinsic coagulation pathway?

    <p>Prothrombin (Factor II)</p> Signup and view all the answers

    What is the primary location of tissue factor (TF) in the body?

    <p>Plasma membrane of many cell types EXCEPT endothelial cells</p> Signup and view all the answers

    Which of the following is NOT a function of phospholipid in the coagulation pathway?

    <p>Inhibiting the fibrinolytic system</p> Signup and view all the answers

    What is the function of Factor XIII in the coagulation pathway?

    <p>To stabilize the fibrin clot</p> Signup and view all the answers

    What is the characteristic of bone marrow in iron deficiency anemia?

    <p>Abundant iron stores in macrophages but not RBC precursors</p> Signup and view all the answers

    What is the challenge in diagnosing iron deficiency anemia in the presence of inflammation?

    <p>Increased serum ferritin levels</p> Signup and view all the answers

    What is the role of sTfR in distinguishing iron deficiency anemia from anemia of chronic inflammation?

    <p>It aids in distinguishing the two conditions</p> Signup and view all the answers

    What is the consequence of accumulating products in the skin in porphyrias?

    <p>Photosensitivity with severe burns on exposure to sunlight</p> Signup and view all the answers

    What is the result of excess porphyrins in the body in porphyrias?

    <p>The excess porphyrins leak from the cells as they age or die and may be excreted in urine or feces</p> Signup and view all the answers

    What is the characteristic of anemia of chronic inflammation?

    <p>Increased serum ferritin levels</p> Signup and view all the answers

    What is the Thomas plot used for in diagnosing iron deficiency anemia?

    <p>To graph hemoglobin content of reticulocytes against sTfR/log ferritin</p> Signup and view all the answers

    What is the consequence of accumulating products in developing teeth and bones in porphyrias?

    <p>Fluorescence of developing teeth and bones</p> Signup and view all the answers

    What is the result of the absence of an enzyme in heme synthesis?

    <p>The products from the earlier stages in the pathway accumulate</p> Signup and view all the answers

    What is the characteristic of only three of the porphyrias?

    <p>Hematologic manifestations</p> Signup and view all the answers

    What is the characteristic morphology of neutrophils in Pelger-Huët Anomaly?

    <p>Pince-nez (Spectacle-like) shape</p> Signup and view all the answers

    What is the significance of X or Sex Chromatin in the nucleus of female cells?

    <p>It is a marker of gene inactivation</p> Signup and view all the answers

    What is the characteristic of Auer rods in myeloid cells?

    <p>They are pink or red-stained needle-like crystals</p> Signup and view all the answers

    What is the function of Faggot cells in the blood?

    <p>They are involved in the immune response</p> Signup and view all the answers

    What is the significance of Pyknotic nucleus in dying neutrophils?

    <p>It is a marker of cell death</p> Signup and view all the answers

    What is the primary defect in MPO deficiency, also known as Alius-Grignaschi Anomaly?

    <p>Absence of MPO enzyme in neutrophils and monocytes but not in eosinophils</p> Signup and view all the answers

    Which of the following diseases is associated with a mutation in the CHS1/LYST gene on chromosome 1q42.1-2?

    <p>Chediak-Higashi syndrome</p> Signup and view all the answers

    What is the characteristic of the lysosomes in neutrophils, monocytes, and lymphocytes in Chediak-Higashi syndrome?

    <p>Abnormal shape and function</p> Signup and view all the answers

    What is a common manifestation of Chediak-Higashi syndrome in infancy?

    <p>All of the above</p> Signup and view all the answers

    What is the primary function of the protein encoded by the CHS1/LYST gene?

    <p>Regulation of lysosomal morphology and function</p> Signup and view all the answers

    What is the primary accumulation in Gaucher's disease?

    <p>Ceramide</p> Signup and view all the answers

    What is the characteristic granulation seen in Alder-Reilly Anomaly?

    <p>Reilly bodies</p> Signup and view all the answers

    What is the primary enzyme deficiency in Niemann-Pick Disease?

    <p>Sphingomyelinase</p> Signup and view all the answers

    What is the primary cell type affected in Alder-Reilly Anomaly?

    <p>All of the above</p> Signup and view all the answers

    What is the primary location of the granules seen in Alder-Reilly Anomaly?

    <p>Cytoplasm</p> Signup and view all the answers

    What type of leukocyte disorder is characterized by the presence of toxic granulation in neutrophils or monocytes?

    <p>Lysosomal Storage Diseases</p> Signup and view all the answers

    What occurs in true anemia?

    <p>Decrease in Red Cell Mass (RCM)</p> Signup and view all the answers

    Which of the following lysosomal storage diseases results from a deficiency of glucocerebrosidase, leading to the accumulation of glucosylceramide in the lysosomes?

    <p>Gaucher's Disease</p> Signup and view all the answers

    What happens to the concentration of RBCs in a dehydrated state?

    <p>It appears elevated</p> Signup and view all the answers

    What is Relative anemia?

    <p>Apparent decrease in RBC count due to increase in plasma volume</p> Signup and view all the answers

    What is the name of the lysosomal storage disease caused by a deficiency of sphingomyelinase, leading to the accumulation of sphingomyelin in the lysosomes?

    <p>Niemann-Pick Disease</p> Signup and view all the answers

    Which of the following morphologic abnormalities is characterized by the presence of small, darkly staining inclusions in the cytoplasm of neutrophils?

    <p>Alder-Reilly Anomaly</p> Signup and view all the answers

    What is the result of increased plasma volume in pregnancy?

    <p>Relative anemia</p> Signup and view all the answers

    What is the term for the abnormal accumulation of lipids within lysosomes, leading to cellular dysfunction and disease?

    <p>Lysosomal Lipid Storage</p> Signup and view all the answers

    What is the difference between absolute anemia and relative anemia?

    <p>Absolute anemia is a true decrease in Red Cell Mass (RCM), while relative anemia is an apparent decrease in RBC count due to plasma volume changes</p> Signup and view all the answers

    What is the effect of hyperproteinemia on RBC count?

    <p>It appears to decrease RBC count due to increased plasma protein levels</p> Signup and view all the answers

    What is the cause of relative erythrocytosis?

    <p>Dehydration</p> Signup and view all the answers

    What is the result of a shift of fluid from extracellular to intracellular?

    <p>Dilution of blood</p> Signup and view all the answers

    What is the characteristic of true anemia?

    <p>True decrease in Red Cell Mass (RCM)</p> Signup and view all the answers

    What is the effect of plasma volume expansion on RBC concentration?

    <p>It appears to decrease RBC concentration</p> Signup and view all the answers

    What is the characteristic of May-Hegglin anomaly?

    <p>An autosomal dominant disorder with variable thrombocytopenia, giant platelets, and large Döhle body-like inclusions</p> Signup and view all the answers

    What is the primary defect in Lysosomal Storage Diseases?

    <p>Flawed degradation of phagocytized material and buildup of undigested substrates within lysosomes</p> Signup and view all the answers

    What is the name of the inclusions seen in May-Hegglin anomaly?

    <p>Myosin inclusions</p> Signup and view all the answers

    What is the genetic mutation associated with May-Hegglin anomaly?

    <p>Mutation in the MYH9 gene on chromosome 22q12-13</p> Signup and view all the answers

    How do Döhle-body-like inclusions in May-Hegglin anomaly differ from Döhle bodies?

    <p>Döhle-body-like inclusions in May-Hegglin anomaly are larger and rounder in shape</p> Signup and view all the answers

    What is the term for the condition characterized by hypersegmentation of neutrophils with more than five lobes or nuclear segments?

    <p>Hypersegmentation</p> Signup and view all the answers

    What is the name of the anomaly characterized by benign, autosomal dominant disorder with decreased nuclear segmentation?

    <p>Pelger-Huët Anomaly</p> Signup and view all the answers

    What is the term for the condition where neutrophils appear to function normally, but with distinctive coarse chromatin clumping pattern?

    <p>Pelger-Huët Anomaly</p> Signup and view all the answers

    What is the underlying genetic mutation responsible for Pelger-Huët Anomaly?

    <p>Mutation in the lamin β-receptor gene</p> Signup and view all the answers

    What is the consequence of homozygous Pelger-Huët Anomaly?

    <p>Cognitive impairment, heart defects, and skeletal abnormalities</p> Signup and view all the answers

    What is the significance of serum ferritin in assessing iron status?

    <p>It reflects the levels of iron stored within cells</p> Signup and view all the answers

    What is the advantage of measuring hemoglobin content of reticulocytes in detecting iron deficiency?

    <p>It is a sensitive indicator of iron deficiency, enabling detection within days</p> Signup and view all the answers

    What is the significance of free erythrocyte protoporphyrin (FEP) levels in iron status?

    <p>They rise when iron is unavailable, forming zinc protoporphyrin (ZPP) when preferentially chelated with zinc</p> Signup and view all the answers

    What is the consequence of iron deficiency on red blood cell production?

    <p>MCH does not change until a substantial proportion of cells are deficient</p> Signup and view all the answers

    What is the significance of sideropenia in iron deficiency anemia?

    <p>It is a condition characterized by decreased serum ferritin and iron levels</p> Signup and view all the answers

    What is the primary effect of chronically elevated hepcidin levels on red blood cell production?

    <p>Diminished production of red blood cells</p> Signup and view all the answers

    What is the primary function of lactoferrin in the context of anemia of chronic inflammation?

    <p>To prevent the use of iron by phagocytized bacteria</p> Signup and view all the answers

    How does hepcidin regulate iron availability during inflammation?

    <p>By sequestering iron in macrophages and hepatocytes</p> Signup and view all the answers

    What is the consequence of lactoferrin release into the blood and extracellular spaces during inflammation?

    <p>Protection of phagocytes from oxidized iron</p> Signup and view all the answers

    What is the primary effect of inflammatory cytokines on hepcidin regulation?

    <p>Stimulation of hepcidin production</p> Signup and view all the answers

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