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 |