von Willebrand's Disease (vWD)

by Carlo Raj, MD

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    00:01 In von Willebrand disease our focus is going to be on, once again, how much of my von Willebrand factor I have present? The combination of platelet function and also what clotting factor? Which one? VIII.

    00:16 The combination of the two is going to give you what test? Good.

    00:21 An increase in bleeding time and an increase in PTT.

    00:25 And here we go.

    00:26 The types.

    00:27 We have type I, mild decrease in von Willebrand factor.

    00:31 Mild.

    00:33 Type II , a defect in von Willebrand factor function but more commonly is the one you're gonna be looking for with complete absense of complete von Willebrand factor.

    00:45 And not only that but you also have lack of stabilization of factor VIII activity.

    00:52 Signs and symptoms include bruising.

    00:56 Remember we talked about that lady who may have inserted a tampon, had a pad on, woke up in the morning and still during her menses are soaking the sheets with blood.

    01:07 Lots of blood everywhere.

    01:09 Bleeding, especially mucous membrane, menorrhagia, unfortunately.

    01:12 At the same time you can also expect there to be iron deficiency, right? Obviously, lots of bleeding taking place.

    01:18 Laboratory test include the following.

    01:21 Predict what's gonna happen first, please? Increase in PTT and increase in bleeding time and especially for type III, abnormal ristocetin cofactor assay.

    01:32 In other words, the ristocetin is then going to cause what's known as aggregation and decreased von Willebrand factor and factor VIII.

    01:42 von Willebrand factors decreased, whereas factor VIII levels are going to be normal in von Willebrand disease type I, whereas both of them are decreased in type III of von Willebrand disease. Ultimately, if you've been following me, you come to this table and everything should speak to you.

    02:04 Laboratory testing for bleeding diathesis.

    02:06 I wanna point out what's important for each one.

    02:09 Pay attention.

    02:10 Aspirin increase in bleeding time, lack of thromboxane, no aggregation, platelet count perfectly normal, no petechia.

    02:20 Thrombocytopenia whatever the cause may be, obviously, decrease in platelet count, increase in bleeding time only.

    02:28 Von Willebrand disease either class or type I and type III.

    02:34 You'd expect there to be an increase in bleeding time because the von Willebrand factor is not functioning properly, a bind to glycoprotein Ib and PTT is elevated due to lack of stabilization of factor VIII.

    02:46 Hemophilia A, let's talk about severe.

    02:50 Not much activity going on with your factor VIII so therefore resulting in increase in PTT.

    02:59 DIC everything's elevated except for platelet count and that'll be decreased.

    03:05 Warfarin and heparin, well you tell me.

    03:09 Warfarin which test are you gonna use? Remember WEPT? Warfarin Extrinsic PT.

    03:16 If it's not warfarin, it's heparin.

    03:18 If it's not extrinsic, it's intrinsic.

    03:21 If it's not PT, PTT.

    03:23 Welcome to heparin.

    About the Lecture

    The lecture von Willebrand's Disease (vWD) by Carlo Raj, MD is from the course Hemostasis: Basic Principles with Carlo Raj.

    Included Quiz Questions

    1. Marked decrease or absence of von Willebrand factor
    2. Moderate decrease of von Willebrand factor
    3. Reduced binding of von Willebrand factor to factor VIII
    4. Reduced binding of von Willebrand factor to glycoprotein Ib
    5. Increased von Willebrand factor clearance
    1. VIII
    2. X
    3. VII
    4. XIII
    5. IX
    1. Bleeding time
    2. Platelet count
    3. White blood cell count
    4. Prothrombin time
    5. Hemoglobin

    Author of lecture von Willebrand's Disease (vWD)

     Carlo Raj, MD

    Carlo Raj, MD

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