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Hematology
FA complete review part 2 Physiology
| Question | Answer |
|---|---|
| Which are the coagulation factors involved in the Intrinsic pathway? | XII, XI, IX, and VIIIa (12, 11, 9, 8a) |
| Deficiency of factor 8 (VIII). Dx? | Hemophilia A |
| Hemophilias represent factor deficiencies in the ______________ coagulation pathway. | Intrinsic |
| Deficiency of factor 9. Dx? | Hemophilia B |
| Deficiency of factor XI. Dx? | Hemophilia C |
| Which is the only hemophilia with AR inheritance mode? | Hemophilia C |
| What is another name for the Extrinsic coagulation pathway? | Tissue factor pathway |
| Which is the coagulation factor involved in the Extrinsic coagulation pathway? | VII (7) |
| Which coagulation factors are involved in the Combined Coagulation pathway? | 10, 5, 2, and 13 |
| 5 x 2 = 10. Easy way to remember what? | Coagulation factors of the Combined pathway |
| Which are the two main factors affected by Anticoagulants? | Factor Xa and Factor IIa (thrombin) |
| Name of coagulation factor IIa? | Thrombin |
| Another way to represent Thrombin? | Factor IIa |
| Which is the anticoagulant affecting Factor Xa with the greatest efficacy? | LMWH |
| Which Factor Xa anticoagulants have the greatest efficacy? | LMWH |
| Which are Direct Xa inhibitors? | Apixaban and Rivaroxaban |
| Which anticoagulant has the greatest efficacy by affecting Factor IIa (thrombin)? | Heparin |
| What are some common LMWH examples? | Dalteparin and Enoxaparin |
| Common direct thrombin inhibitors: | Argatroban, bivalirudin, and dabigatran. |
| What part of the Coagulation cascade do Anticoagulants work on? | Combined Coagulation pathway |
| What are effects caused by Bradykinin? | 1 . Increased vasodilation 2. Increased permeability 3. Increased pain |
| Which coagulation factors are inhibited by Warfarin? | 10, 9, 7, and 2 |
| Which coagulation factors are considered "cofactors"? | 8a and 5a |
| What activates Bradykinin? | Kallikrein |
| What enzyme inactivates bradykinin? | ACE |
| Thrombolytics work on the _______________ ______________. | Fibrinolytic system |
| What are common thrombolytics? | Alteplase, reteplase, streptokinase, and tenecteplase |
| Thrombolytics stimulate the action of _________. | tPA |
| What acid is known to inhibit tPA? | Aminocaproic acid |
| Plasminogen + tPA -----> | Plasmin |
| What is the role of Plasmin? | Breakdown fibrin platelet plugs |
| What factors and protein decrease synthesis in Vitamin K deficiency? | Factors II, VII, IX, X, and protein C and protein S. |
| What enzyme is inhibited by Warfarin? | Vitamin K epoxide reductase |
| Why are neonates given a vitamin K injection? | Neonates lack enteric bacteria, which produce vitamin K |
| Which factor has the shortest half life? | Factor VII (7) |
| Which coagulation factor has the longest half life? | Factor II |
| Which factor carries/protects vWF? | Factor VIII (8) |
| What factors are inactivated by antithrombin? | 2, 7, 9, 10, 11, and 12 |
| Which anticoagulant enhances the activity of antithrombin? | Heparin |
| What are the principal targets of antithrombin? | Thrombin and factor Xa |
| What is produced by a Factor V Leiden mutation? | Factor V resistant to inhibition by activated protein C |
| What is the clinical use for tPA? | Thrombolytic |
| Fibrinolysis consists of: | 1. Cleavage of fibrin mesh 2. Destruction of coagulation factors |
| Activated Protein C + Protein S ===> | Cleaves and inactivates Va, VIIIa |
| Primary hemostasis refers to: | Platelet plug formation |
| What is the first step in Platelet plug formation? | Injury - Endothelial damage |
| Transient vasoconstriction via neural stimulation reflex and endothelin. | Step 1 in Platelet plug formation (endothelial damage) |
| What step in platelet plug formation consists of vWF binding to exposed collagen ? | Step 2 (exposure) |
| vWF originates from? | Weibel-Palade bodies of endothelial cells and alpha-granules of platelets. |
| What is the product of Weibel-Palade bodies of endothelial cells and alpha-granules of platelets? | vWF |
| Adhesion is step ______ in the platelet plug formation process. | 3 |
| Platelets binding to vWF via GpIb receptor at site of injury only. | Step 3 in Platelet plug formation |
| Immediate result of platelet binding to vWF via GpIb receptor? | Release of ADP and Ca2+, and TXA2 |
| What happens once ADP binds to P2Y12 receptor? | Induces GpIIb/IIIa expression at platelet surface |
| What is the initial step in "aggregation" of platelet plug synthesis process? | Fibrinogen binds GpIIb/IIIa receptors and links platelets |
| What are Pro-aggregation factors? | 1. TXA2 (released by plts) 2. Decreased blood flow 3. Increase platelet aggregation |
| Anti-aggregation factors: | 1. PGI2 and NO (released by endothelial cells) 2. Increased blood flow 3. Decreased platelet aggregation |
| Formation of insoluble fibrin mesh | Thrombogenesis |
| Which pain medication irreversibly inhibits COX, causing inhibition of TXA2 synthesis? | Aspirin |
| Aspirin interferes with the process of _____________________. | Thrombogenesis |
| Which drugs irreversibly block P2Y12 receptor? | Clopidogrel, prasugrel, and ticlopidine |
| What is the result of blocking P2Y12 receptor? | ADP-induced expression of GpIIb/IIIa |
| Drugs that directly inhibit GpIIb/IIIa receptor? | Abciximab, eptifibatide, and tirofiban |
| Abciximab directly inhibits _________________ receptor. | GpIIb/IIIa |
| What is activated by Ristocetin? | vWF to bind GpIb |
| Which conditions present with failure of aggregation with ristocetin assay? | 1. von Willebrand disease and, 2. Bernard-Soulier syndrome |
| vWF deficiency. Dx? | von Willebrand disease |
| GpIb deficiency. Dx? | Bernard-Soulier syndrome |
| vWF attaches to the __________________________. | Subendothelial collagen |
| GpIb attaches the ____________________ to the __________. | Platelet to the vWF. |
| If in VWD, there is a vWF deficiency it means? | No platelet attachment to the subendothelial collagen |