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60 Cards in this Set
- Front
- Back
The ability to maintain blood in a fluid state and prevent loss from sites of vascular damage
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Hemostasis
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3 components of the hemostatic system
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1) the vascular wall
2) platelets 3) coagulation proteins |
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Primary hemostatic plug
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The excitation of platelets due to the sub endothelial collagen exposure after injury
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Platelet response to vascular injury is ___hemostasis
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Primary
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Platelet adhesion involves activation of its membrane receptor ___, the adhesion protein _____, and the appropriate surface of ____
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Glycoprotein Ib/IX, von Willebrand factor, subendothelial collagen
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Mediates the adhesion of platelets to collagen
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von Willebrand factor
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Which membrane receptor becomes activated during the recruitment of other platelets?
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Glycoprotein IIb/IIIa
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What happens during the activation phase of the primary hemostatic response?
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1) Release of 2nd messengers
2) Secretion of ADP 3) Activation of IIb/IIIa receptor |
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What causes platelets to adhere to one another?
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Cytoplamic ADP
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Fibrin clot formation is ___hemostasis
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Secondary
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What does thrombin do?
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Converts fibrinogen to fibrin, which adds stability to the blood clot
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How are fibrin monomers linked?
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Covalently by Factor XIII
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The activation of factor XII by kallikrein, followed by activation of Factor XI by XIIa; factor XIa activates factor IX. This is which pathway?
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The intrinsic pathway
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The activation of factor VII by tissue factor is by which pathway?
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The extrinsic pathway
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This pathway involves the activation of factor X to Xa, followed by conversion of pro thrombin to thrombin and fibrinogen to fibrin.
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The common pathway
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Primary regulatory hemostasis regulates what?
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Platelets
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What inactivates Factor X?
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Antithrombin
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Antithrombin pairs with ___to inactivate thrombin
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Heparin
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A major effector enzyme that regulates the cofactors Va and VIIIa
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Protein C
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A major cofactor that helps in secondary hemostasis
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Protein S
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This binds to plasminogen and turns it into plasmin
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Tissue plasminogen activator (TPA)
- can be used in patients who have had MI |
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What does plasmin do?
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Breaks up fibrin clots
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Shifts that favor the regulation steps lead to _____
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Bleeding disorders
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Shifts that favor pro coagulation result in ____
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Thrombosis
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A measurement of the time needed for plasma to form a clot in the presence of added tissue thromboplastin and Ca2+ ions
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Prothrombin time (PT)
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Routinely used to measure degree of anticoagulation in patients receiving oral anticoagulants
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PT
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Prolonged PT can result from what?
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- Decreased/abnormalities in Factor II, V, VII, X and/or fibrinogen
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A measurement of the time needed for plasma to form a clot in the presence of added kaolin (to activate contact-dependent factor XII), cephalin (phospholipid), and Ca2+ ions
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Partial thromboplastin time (PTT)
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PT initiates this coagulation pathway
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Extrinsic
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PTT activates this pathway
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Intrinsic
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Prolonged PTT can result from what?
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Decreased or abnormalities in factors II, V, VIII, IX, X, XI, XII and fibrinogen
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Routinely used to measure degree of anticoagulation in patients receiving heparin therapy
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PTT
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Normal range of platelets
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150,000-400,000/ uL
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Thrombocytosis and thrombocythemia denote what?
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Increase in platelet number
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A decrease in platelet number
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Thrombocytopenia
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This procedure, which replaces bleeding time, performs like in vitro bleeding time
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PFA-100
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If a mixing study corrects clotting time, what was deficient?
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A factor(s)
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If mixing study doesn't correct clotting times, what does this signify?
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An inhibitor is present, either factor-specific or lupus anticoagulant
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Acquired bleeding disorders are often caused by what?
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Medication or a pathological process
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Clinical manifestations include mucocutaneous bleeding and/or bleeding associated with trauma. Laboratory manifestations include a prolonged bleeding time and thrombocytopenia.
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Disorder of primary hemostasis
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Clinical manifestations include soft tissue bleeding and/or bleeding associated with trauma. Laboratory manifestations include a prolonged PT and/or PTT and/or thrombin time.
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Disorders of secondary hemostasis
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Clinical manifestations include soft tissue bleeding and/or bleeding associated with trauma. Laboratory manifestations are often normal in screening tests.
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Disorders of the regulatory system
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von Willebrand factor is a carrier molecule for what factor?
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Factor VIII
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Different epitopes on vWF are responsible for what?
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Its adhesion to endothelium and platelets and it association to factor VIII
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This factor participates in the generation of Factor X
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Factor VIII
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T/F, Factor VIII is released from vWF when a clot is formed?
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True
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Where is Factor VIII synthesized?
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The liver
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This is most often an autosomal dominant disorder associated with production of decreased amounts of a normal protein (quantitative abnormality) or production of a protein with abnormal function (qualitative abnormality), or both. The dominant clinical manifestation is mucocutaneous bleeding
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vWF Syndrome
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Clinical manifestations of vWF are?
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Laboratory manifestations include prolonged bleeding time, prolonged PTT (due to decreased Factor VIII), and decreased vWF and decreased Factor VIII
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Therapeutic options for vWF syndrome are
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desmopressin (releases vWF from endothelial cells) and antifibrinolytic agents in mild cases, factor VIII concentrates containing vWF and cryoprecipitate in more severe cases
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This is a sex-linked recessive disorder that is due to decreased production of Factor VIII. The clinical hallmark is recurrent soft tissue bleeding, and symptoms usually start early in life. Clinical manifestations include hemarthrosis, soft tissue bleeding, bleeding with trauma, intramuscular hematomas, intracranial hemorrhage, and generally excessive bleeding
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Hemophilia A
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Laboratory features of hemophilia a include what?
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Laboratory features include normal bleeding time, prolonged PTT, decreased VIII, normal vWF, and normal IX. Severe hemophilia A is characterized by a Factor VIII level of <1%, moderate forms have 1-5% VIII, and mild forms have >5% VIII
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Decreased factor IX will lead to this disorder
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Hemophilia B
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Deficiencies in these will give markedly prolonged PTT's, but these patient's aren't at increased risk for bleeding
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Factor XII and contact factors
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Patients w/ deficiencies in this will have inc. risk for bleeding and have normal PT and PTT levels
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Factor XIII
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Bleeding due to thrombocytopenia presents as what?
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Petechial hemorrhages in skin and mucous membranes
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Spontaneous bleeding due to thrombocytopenia is seen when cells are below what number?
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20,000
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This disorder is often an immune mediated destruction of platelets. Platelets coated with antibody directed against platelet-specific antigens are sequestered in the spleen and reticuloendothelial system
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Idiopathic Thrombocytopenia Purpura (ITP)
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This is an acute disorder of unknown cause that is characterized by intravascular platelet activation with formation of platelet-rich microthrombi throughout the circulation. Left untreated, it is associated with high mortality
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Thrombotic Thrombocytopenia Purpura (TTP)
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This disorder results from uncontrolled activation of the hemostatic system. There is both systemic thrombin formation and systemic plasmin formation
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DIC
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