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hemostatic disorders
handbook
| Question | Answer |
|---|---|
| Clinical effects of Factor XII deficiency | none |
| Describe the clinical bleeding associated with von Willebrand's disease | mucosal bleeding(epistaxis, menorrhagia, GI bleed), excessive bruising, or bleeding after minor trauma |
| Describe the clinical bleeding associated with platelet disorders | mucosal bleeding(epistaxis, menorrhagia, GI bleed), excessive bruising, or bleeding after minor trauma |
| Describe the bleeding associated with hemophilia | Bleeding into joints, muscles or soft tissues |
| The most common causes of primary fibrinolysis | Severe liver disease and hepatic surgery |
| In non-bleeding patients with thrombocytopenia caused by marrow failure, prophylactic platelet transfusions are generally reserved for platelet counts less than this number | 10K |
| This syndrome is due to deficiency of GPIb/IX in platelets | Bernard-Soulier |
| This syndrome is due to deficiency of GPIIb/IIIa in platelets | Glanzmann thrombasthenaa |
| This drug has been effective treating bleeding associated with uremia | DDAVP(desmopressin) |
| Describe the underlying pathophysiology of the most common type of von Willebrand's disease | Type 1 vWD has mild to moderate quantitative deficiency of vWF |
| In this type of von Willebrand's disease, vWF is virtually absent along with Factor VIII | Type 3 vWD |
| These lysine analogues are used as anti-fibrinolytics | epsilon aminocaproic acid and tranexamic acid |
| Mechanism of action of tranexamic acid and epsilon aminocaproic acid | interfere with plasmin binding to fibrin, thereby inhibiting fibrinolysis (anti-fibrinolytics) |
| This site of bleeding is a contraindicationto tranexamic acid and EACA | upper urinary tract bc obstruction may result from failure of clot lysis |
| X-linked congenital bleeding disorder caused by Factor VIII deficiency | Hemophilia A |
| Products of choice in Hemophilia A | Recombinant Factor VIII |
| FDA requires that each unit of cryoprecipitated AHF contain this much factor VIII | 80 IU |
| DDAVP and cryo can be useful in the treatment of: A. Hemophilia A B. Hemophilia B C. Both D. Neither | A. |
| What is the mainstay of therapy for hemophilia patients with inhibitors to factor VIII or IX? | activated Prothrombin complex concentrates (aPCC) or recombinant Factor VIIa (rFVIIa) |
| Treatment of choice for Factor V deficiency | plasma |
| Treatment for Factor XII deficiency | none |
| This deficiency is associated with umbilical stump bleeding | Factor XIII |
| Treatment for alpha2-plasmin inhibitor deficiency | epsilon aminocaproic acid (EACA) |
| Vitamin K dependent coagulation factors | II,VII,IX, X |
| Warfarin-induced skin necrosis is related to the drug's early effect on this | protein C synthesis |
| If patient has serious bleeding on warfarin, this product is recommended | 4-factor (II,VII,IX,X) Prothrombin complex concentrate (PCC) |
| Calculation for FFP units needed to reverse heparin effect | plasma is not effective in reversing heparin effect |
| Drug used to reverse heparin effect | protamine sulfate |
| This drug is a synthetic pentasaccharide that strongly binds anti-thrombin and acts as a selective inhibitor to Factor Xa | Fondaparinux |
| This drug is an oral targeted thrombin inhibitor | Dabigatran (Pradaxa) |
| These drugs are oral targeted Factor Xa inhibitors | Rivaroxaban (Xarelto) and Apixaban (Eliquis) |
| These drugs selectively inhibit ADP-induced activation of platelets via irreversible alteration of the platelet P2Y12 receptor | Clopigrodel (Plavix) and Prasugrel (Effient) |
| This is a synthetic analogue of vasopressin | DDAVP |
| These factors are released from endothelial cells when DDAVP is given | vWF, Factor VIII, and tPA |
| These antifibrinolytic drugs are synthetic analogues of lysine | EACA and Tranexamic acid |
| Mechanism of action of tranexamic acid | occupies lysine binding sites of plasminogen and plasminogen activators which delays clot resorption |
| This is the most important protease inhibitor of activated coagulation factors and is central to the in-vivo effect of heparin | antithrombin |
| Condition in infants with homozygous deficiency of protein C or S | purpura fulminans |
| When does "warfarin induced skin necrosis occur"? | When patients with protein C or S deficiency are started on warfarin |
| This is the specific antibody epitope in anti-phospholipid syndrome | beta-2 glycoprotein I |
| Clinical effect of "Lupus anti-coagulant" | thrombosis |
| Lab criteria for Lupus anticoagulant | 1. prolonged PTT 2. prolonged Russell viper venom test 3. fails to correct on 1:1 mixing study 4. corrects when excess phospholipid is added |
| This is a drug associated ITP disorder characterized by paradoxical thrombosis | HIT: heparin induced thrombocytopenia |
| These tests are used to confirm a dx of HIT | antibodies to platelet factor 4/heparin complexes or serotonin release assay |
| Platelet dose for a patient with HIT and a platelet count of 19,000 | none. avoid platelet transfusion in this dx |
| The majority of adult-onset cases of TTP have a deficiency of this protein | ADAMTS13 |
| Outline treatment of TTP if TPE is not readily available | FFP at 40ml/kg until TPE can be arranged |
| Etiology of Typical hemolytic uremic syndrome (HUS) | shiga toxin producing E. coli |
| Best treatment for atypical HUS | eculizamab, C5 monoclonal antibody, disease results from excessive activation of alternative complement pathway |