Q1 Coagulation

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Q1 Coagulation
2010-10-02 17:07:19
Q1 Coagulation Heme Onc

Q1 Coagulation Heme/Onc
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  1. Platelets bud off of what part of megakaryocyte
  2. Growth factor required for platelet maturation
  3. How long do platelets circulate in the blood?
    7 days
  4. Do platelets have nuclei?
  5. Contents of α granules
    • Fibrinogen
    • vWF
  6. Contents of dense granules
    ADP, Ca2+
  7. Granules contained in platelets (2)
    • α granules
    • Dense granules
  8. Other name for Giant Platelet Syndrome
    Bernard-Soulier syndrome
  9. What does platelet function analysis (PFA-100) measure?
    Closure time (measures primary hematostasis)
  10. Low platelet count and value of low platelet count
    • Thrombocytopenia
    • <150 X 10^9
  11. High platelet count and value considered high platelet count
    • Thrombocytosis
    • >450 X 10^9
  12. Elevated platelet count in context of pathological myelproliferation
  13. Myeloproliferative disorders associated with thrombocythemia
    • Essential thrombocythemia (>600)
    • Polycythemia vera
    • Myelofibrosis with myeloid metaplasia
  14. ITP
    Immune thrombocytopenia purpura
  15. Autoimmune diseases associated with thombocytopenia (2)
    • ITP
    • Lupus
  16. Conditions associated with intravascular overconsumption of platelets
    • DIC
    • TTP
    • HUS
  17. Condition associated with extravascular consumption of platelets
    Hyperspleenism (platelets and blood pool in spleen)
  18. Congential bone marrow failure syndromes (leading to thrombocytopenia)
    • Fanconi's anemia
    • Schwachman-Diamond syndrome
  19. Condition associated with defect in DNA repair, leading to pancytopenia
    Falconi's anemia
  20. Bone marrow failure syndrome associated with pancreatic insufficiency
    Schwachman-Diamond Syndrome
  21. CAMT
    Congenital Amegakaryocytic Thrombocytopenia
  22. Conditions associated with genetic mutation of thrombopoietin receptor gene
    Congential Amegakaryocytic Thrombocytopenia (CAMT)
  23. Condition associated with small platelets and thrombocytopenia
    Wiskott-Aldrich syndrome
  24. Condition where there are antibodies to platelet receptors
    Immune Thrombocytopenic Purpura
  25. Condition with microclots seen in small vessels where micro-clots are non-occlusive
    • Microangiopathic Hemolytic Anemia (MAHA)
    • RBCs get tangled in strands
  26. Conditions associated with intravascular consumption --> thrombocytopenia
    • FIC
    • TTP/HUS
  27. Ig associated with ITP
  28. Platelet receptor attacked in ITP
  29. Systemic microangiopathic hemolytic anemia (MAHA) resulting in ischemia of organs
    Thrombotic thrombocytopenic purpura
  30. Enzyme lacking in congenital TTP
  31. TTP diagnostic pentad
    • Thrombocytonpenia
    • MAHA
    • Fever
    • Neurological abnormalities (MAHA occurring in brain)
    • Renal failure (MAHA occurring in kidneys)
  32. Enzyme that cleaves vWF
  33. Consequence of deficiency of ADAMS13
    • Large uncleaved vWF
    • excessive adhesion --> clotting complications
  34. Condition with thrombocytopenia, MAHA, and acute renal failure
    Hemolytic Uremic Syndrome (HUS)
  35. Hypercoagulable state resulting from idiosyncratic reaction to heparin
    Heparin induced thrombocytopenia
  36. Ig associated with heparin-induced thrombocytopenia and the platelet antigen bound to heparin
    • IgG
    • Platelet factor 4
  37. Deficiency in Bernard-Soulier syndrome
    Glycoprotein Ib-IX (platelet can't adhere to subendothelium)
  38. Aggregation defect due to lack of binding with fibrinogen
    Glanzmann's Thrombasthemia
  39. Function of glycoprotein IIb-IIIa in platelet aggregation
    Cross-linking of fibrinogen
  40. Defect in Glanzmann's Thrombasthemia
    Glycoprotein IIb-IIIa receptor
  41. Condition associated with α granule deficiency
    Gray platelet syndrome
  42. Condition associated with dense granule deficiency
    Wiskott-Aldrich syndrome
  43. TTP vs. HUS with respect to fibrogenemia
    • TTP: hypofibrigenemia possible
    • HUS: no hypofibrogenemia (localized to kidney)
  44. Difference between aspirin and other NSAIDs with respect to platelet inhibition
    • Aspirin: Irreversible
    • Other NSAIDs: Reversible
  45. Penicillin derivative that can cause dose-related platelet dysfunction
  46. Drugs besides aspirin that cause platelet dysfucntion
    • pencillin derivatives
    • Vaproic acid
  47. Drug that causes fight or flight response, leading to release of vWF and factor VIII
    Desmopressin (DDAVP)
  48. Drug that enhances the clotting process by inhibiting fibrinolysis
    Aminocaproic acid
  49. Tissue factor is associated with which coagulation pathway?
  50. Factor VII is associated with which coagulation pathway?
  51. Coagulation pathway that INITIATES the process of clot formation
  52. Coagulation pathway that CONTINUES the process of clot formation
  53. Factors (2) that contribute to prothrombin being cleaved into thrombin
    • Xa
    • Va
  54. Thrombin cleaves which factors?
    • XIII
    • fibrinogen
    • V
    • VIII
  55. Hemophilia A is deficiency in factor ___
  56. Hemophilia B is a deficiency in factor _____
  57. Hemophilia C is a deficiency in factor ____
  58. Factors for which a deficiency would lead to both elevated PT and PTT
    • Thrombin (factor II)
    • X
    • V
  59. Treatment used on battlefield to stop heavy bleeding
    Synthetic factor VII
  60. Normal PT and normal PTT
    • PT: 12-15 sec
    • PTT: 25-39 sec
  61. Factor VIII cleaves what factor?
  62. What effect would vitamin K deficiency have on PT and PTT
    Both increased
  63. PT
    Prothrombin time
  64. PTT
    Partial thromboplastin time
  65. Time needed for coagulation pathway to yield end products
  66. What does thrombin time measure?
    Prothrombin conversion to thrombin and fibrinogen coversion to fibrin
  67. If thrombin time elevated, what is the likely cause?
    Abnormal fibrinogen
  68. Effect of heparin on thrombin time, PT, PTT
    • Thrombin: Increased
    • PT: Can be increased
    • PTT: increased
  69. Effect of liver dysfuction on thrombin time, PT, PTT
    • Thrombin time: Increased
    • PT: normal
    • PTT: increased
  70. Trade name of warfarin
  71. Effect of warfarin on PT
  72. Effect of von Willebrand's disease on PTT
    Usually elevated, but not always
  73. What do you suspect if you have elevated PT, PTT and also thrombocytopenia?
    Consumptive coagulatopathy (DIC, Kasabach-Merrit syndrom)
  74. 2 substances assoicated with regulation of fibrinolysis
    • α2 antiplasmin
    • Plasminogen activator inhibitor-1
  75. What does protein C inhibit?
  76. Mutation on active site of factor V where protein C can't bind and therefore can't apply breaks to clot formation
    Factor V Leiden mutation
  77. What 2 products does antithrombin III inhibit
    • Xa
    • Thrombin
  78. What will a deficiency in antithrombin III cause?
  79. What compound does heparin enhance?
    Anti-thrombin III
  80. What would happen if anti-thrombin III deficient patient is given heparin?
    Heparin would be useless
  81. Enzyme that drives conversion of homocysteine into methionine
  82. Consquence of mutation MTHFR
    Back-up of homocysteine--> blood vessels injured-->endothelial cells prothrombotic
  83. Antibody which causes lab anomaly which procudes elevated PTT
    Lupus anticoagulant (antibody)
  84. Auto-immune syndrome that causes antibodies vs. phospholipids, possibly increasing risk of thrombus
    Antiphospholipid syndrome
  85. Hyperhomocysteinemia and antiphospholipid syndrome associated with what kind of thrombi: arterial or venous?
  86. vWF is a carrier protein for what factor?
    VIII (released when vWF binds subendothelium)
  87. Treatment for von Willebrand's disease?
    Desmospressin (DDAVP)
  88. Differences in types of bleeding with von Willebrand's disease and hemophilia
    • von Willebrand: superficial, mucocutaneous
    • Hemophilia: deep tissues
  89. PTT elevation difference in Hemophilia and von Willebrand
    • von Willebrand: 2-3 seconds prolonged
    • Hemophilia: severly prolonged
  90. What does D-dimer measure?
    • Measures end product of clotting
    • Increased D-dimer can indicate thrombotic event
  91. Syndrome where left iliac vein compressed by right iliac artery
    • May-Thurner
    • Prone to clot formation
  92. Difference between heparin and tPA in clot treatment
    • Heparin: prevents additional clot, but doesn't breakdown already existing clot
    • tPA: breaks down existing clot, but doesn't prevent additional clots
  93. Factors included in FFP
    • Fibrinogen
    • vWF
    • XIII
    • VII
  94. Risk factors for arterial thrombotic events
    • Hyper-homocysteinemia
    • Lipoprotein A
    • Anti-phospholipid antibody syndrome
  95. Function of aminocaproic acid
    Enhances platelet function
  96. NATP
    • Neonatal alloimmune thrompocytopenia
    • Mother makes antibody vs fetal platelet antigen inherited from father
  97. Possible causes of purpura fulminans (purpura + DIC)
    • Deficiency in protein C or S
    • Infection with gram neg bacteria (especially meningococcus)
  98. Organized meshwork of thin-walled capillary venous sinuses with surrounding extracellular matrix
    Bone marrow
  99. Differentiate between primary and secondary hemostasis
    • Primary: platelet activation and aggregation--> plug
    • Secondary: Coagulation cascade --> fibrin clot
  100. Event that activates extrinsic cascade
    Tissue factor exposed on inured endothelial cells
  101. Factors in intrinsic pathway
    • 12
    • 11
    • 9
    • 8
  102. Factors in extrinsic pathway
  103. Factors in common pathway
    • 10
    • 5
    • 2 (thrombin)
  104. Hemocrit value relative to hemoglobin value
    Hemocrit should be 3x higher than hemoglobin
  105. Measurement of the integrity of the extrinsic and final common pathways
    Prothrombin time (PT)
  106. Time (in seconds) for patient's blood to clot after addition of Ca2+ and activator of extrinsic pathway (thromboplastin)
  107. Activator of extrinsic pathway in PT test
    Thromboplastin (tissue factor + phospholipids)
  108. INR
    • International normalized ratio
    • Standardizes PT due to differences in thromboplastin sensitivities
  109. Measures the integrity of the intrinsic and final common pathways
    activated partial thromboplastin time (aPTT)
  110. Contents of partial thromboplastin
    Phospholipid only (no tissue factor)
  111. Time for patient plasma to clot after addition of phospholipid and Ca2+
  112. Coaguation confounding factors (3)
    • High hematocrit (citrate anticoagulant concentration too high for plasma volume)
    • Small blood sample size *citrate anticoagulant concentration too high for plasma volume)
    • Sample opacity
  113. Antiphospholipid antibodies can prolong ____, but are actually associated with _____
    • aPTT
    • Thrombosis
  114. What is mixed in a mixing study?
    • Blood sample that showed elevated PT or PTT mixed with normal plasma
    • If factor deficiency, PT and PTT will return to normal
    • If inhibitor, PT and PTT will still be abnormal
    • (lupus anticoagulant is considered an inhibitor)
  115. Vitamin K dependent factors
    • II (thrombin)
    • 7
    • 9
    • 10
  116. Factors made in the liver
    • II (thrombin)
    • 7
    • 9
    • 10
    • 5
    • ** all are vitamin K dependent except for 5
  117. Condition where PT or INR prolonged and factor V depleted
    Synthesis defect in liver
  118. Target of warfarin
    Vitamin-K dependent factors
  119. Deficient factor in coagulation cascade with amyloidosis
    • Factor X
    • Protein fibrils will adhere to factor X
  120. Deficient factor in coagulation cascade with myeloproliferative disease
    Factov V
  121. Deficient factors in von Willebrand syndrom (2)
    • von Willebrand factor
    • Factor VIII
  122. Deficiencies in these factors cause NO bleeding risk (3)
    • Factor XII
    • Kininogen
    • Prekallikrien
  123. How is undfractionated heparin administered?
    Continuous drip IV
  124. How is low molecular weight heparin usd?
    2x/day subcutaneous
  125. How do you reverse unfractionated heparin?
  126. How is low molecular weight heparin administered?
    Weight-based subcutaneous injection
  127. How do you reverse low molecular weight heparin?
    Can only partially reverse with protamine
  128. What medication works as a vitamin K antagonist?
    Warfarin (Comoudin)
  129. How is warfarin administered?
  130. How do you reverse warfarin?
    Vitamin K and fresh frozen plasma (replaces factors that are lost)
  131. VKA
    Vitamin K antagonist
  132. When is oral vitamin K treatment vs. IV vitamin K treatment used
    • If INR high and there is bleeding, give vitamin K in IV
    • Otherwise, give oral vitamin K to replenish
  133. Forms of heparin-induced thrombocytopenia
    • Type 1: non-immune (clumping sequestration), can continue with heparin (no risk of thrombosis)
    • Type 2: allergic reaction to heparin
  134. How are platelets activated in type II heparin-induced thrombocytopenia?
    Auto-antibodies link heparin and platelet factor 4 --> platelet activation --> thrombus formation
  135. Types of Type II heparin-induced thrombocytopenia
    • Early onset (exposed to heparin within 3 months --> formation of anti-heparin antibodies have formed)
    • Delayed (days or weeks after stopping therapy)
  136. When does delayed onset heparin induced thrombocytopenia manifest?
    Days to weeks after discontinuation of heparin therapy
  137. Risk of thrombus if you have heparin-induced thrombocytopenia type II
    30-fold increase in risk (even if no thrombus forms, GIVE ANTICOAGULANTS - direct thrombin inhibitor usually used)
  138. Methods for testing for heparin-induced thrombocytopenia
    • 1. Serologic assay to test for circulating IgG, IgM, IgA heparin-dep antibodies
    • 2. If serologic assay positive, do functional assay to detect heparin-dep antibodies that can react with Fx receptors on platelets
  139. What happens if you give warfarin when the patient has active HIT-2?
    • Warfarin-induced skin necrosis
    • Venous gangrene in limbs
    • (wait until platelets come back)
  140. 3 major mechanisms of DIC
    • Excessive generation of thrombin from increased levels of tissue factor
    • Impairment of normal anticoagulation (deficiencies in ATIII, protein S, protein C)
    • Sustained release of PAI-1 -> suppression of fibrinolysis
  141. Degredation product of plasmin-mediated proteolysis of cross-linked fibrin
  142. Compounds which will prolong PTT in deficiency but are not associatd with bleeding risk
    • Factor XII
    • Kininogen
    • Prekallikrein
  143. Target of low molecular weight heparin
  144. Action of direct thrombin inhibitor
    Binding and inactivating thrombin without a cofactor (e.g. antithrombin)
  145. Conditions that SHORTEN PTT
    • Elevation of VIII
    • Suboptimal sample collection
  146. What kind of heparin in contraindicated for those with renal disease?
    Low molecular weight heparin
  147. Anticoagulant particular susceptible to drug-drug interaction
  148. INR goals in VKA therapy for people with diagnosed AMI vs. people with high risk of AMI
    • Diagnosed AMI: higher goal
    • Increased risk: lower goal
  149. DIC treatment
    • Treat underlying condition
    • Replace factors lost only if significant bleeding
    • Anticoagulation only when coagulation is excessive