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) (D)</p> Signup and view all the answers

What is the function of prekallikrein in the coagulation pathway?

<p>Acceleration of factor XII activation (A)</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 (C)</p> Signup and view all the answers

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

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

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

<p>Factor XIIa (A)</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 (B)</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 (A)</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 (D)</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 (A)</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 (A)</p> Signup and view all the answers

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

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

Which factor is responsible for initiating the intrinsic coagulation pathway?

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

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

<p>Platelet function (A)</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 (D)</p> Signup and view all the answers

What is the primary function of the contact group?

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

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

<p>Factor IX (C)</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 (D)</p> Signup and view all the answers

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

<p>Liver (D)</p> Signup and view all the answers

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

<p>Plasmin (C)</p> Signup and view all the answers

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

<p>Fibrinogen (C)</p> Signup and view all the answers

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

<p>Inhibition of plasmin activity (D)</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 (D)</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 (D)</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 (A)</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 (D)</p> Signup and view all the answers

What is the primary function of the fibrinolytic system?

<p>Breakdown of fibrin clots (B)</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 (C)</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) (B)</p> Signup and view all the answers

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

<p>Factor XI (A)</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 (C)</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 (C)</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) (C)</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 (A)</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 (A)</p> Signup and view all the answers

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

<p>To stabilize the fibrin clot (A)</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 (D)</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 (B)</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 (C)</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 (B)</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 (D)</p> Signup and view all the answers

What is the characteristic of anemia of chronic inflammation?

<p>Increased serum ferritin levels (B)</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 (A)</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 (D)</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 (A)</p> Signup and view all the answers

What is the characteristic of only three of the porphyrias?

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

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

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

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

<p>All of the above (D)</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 (C)</p> Signup and view all the answers

What is the primary accumulation in Gaucher's disease?

<p>Ceramide (B)</p> Signup and view all the answers

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

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

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

<p>Sphingomyelinase (B)</p> Signup and view all the answers

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

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

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

<p>Cytoplasm (C)</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 (D)</p> Signup and view all the answers

What occurs in true anemia?

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

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

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

What is Relative anemia?

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

What is the result of increased plasma volume in pregnancy?

<p>Relative anemia (A)</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 (C)</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 (B)</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 (A)</p> Signup and view all the answers

What is the cause of relative erythrocytosis?

<p>Dehydration (B)</p> Signup and view all the answers

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

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

What is the characteristic of true anemia?

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

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

<p>Myosin inclusions (A)</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 (D)</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 (C)</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 (D)</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 (B)</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 (D)</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 (C)</p> Signup and view all the answers

What is the consequence of homozygous Pelger-Huët Anomaly?

<p>Cognitive impairment, heart defects, and skeletal abnormalities (B)</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 (A)</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 (A)</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 (C)</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 (B)</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 (C)</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 (D)</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 (A)</p> Signup and view all the answers

How does hepcidin regulate iron availability during inflammation?

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

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

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

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