by Carlo Raj, MD

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    00:01 If it is hemophilia. Please note as being X-linked deficiency of, if its A, then it'll be hemophilia A with Factor VIII deficiency.

    00:12 VIII comes before IX, A comes before B, if Factor IX is deficient then we have hemophilia B and as I was making fun of earlier, we're doctors so we don't get 12 days of Christmas, we only get 9.

    00:29 The reason I bring this to your attention is because Factor IX deficiency is also called Christmas disease.

    00:36 Now, the severity of the disease, this is how you read the table.

    00:40 What this table is telling you is what is the percentage of my factor? For example, Factor VIII, present or not? If it's severe, less than 1% of the Factor VIII is active, basically you have no Factor VIII activity.

    01:01 Remind me once again, in both of these, which test will be elevated? PTT.

    01:09 What's my time in PTT? Partial thromboplastin time.

    01:14 25 to 40 seconds.

    01:16 How many times have we discussed that? All about repetition.

    01:20 Moderate 1 to 5%, and mild would be, look at that, you may have up to 50% activity.

    01:26 So therefore there, the hemarthrosis would not be seen, less likely to find hemoarthrosis.

    01:32 What about hemarthrosis? What are you looking for? Well, we'll talk about the severe first.

    01:38 Massive bleeding as we should do in surgery, spontaneous hemarthrosis, bleeding into your joints.

    01:44 We talked about PTT, elevated only, because both VIII and IX, only part of intrinsic, PT will be normal, obviously bleeding time has nothing to do with this because these are coagulation factor issues.

    01:56 Treated with recombinant, either VIII or IX. Quite effective.

    About the Lecture

    The lecture Hemophilia by Carlo Raj, MD is from the course Hemostasis: Basic Principles with Carlo Raj.

    Included Quiz Questions

    1. 1-5% of normal
    2. 5-40% of normal
    3. < 1% of normal
    4. 80%-99% of normal
    5. 50-80% of normal
    1. Partial thromboplastin time
    2. Bleeding time
    3. International normalisation ratio
    4. Prothrombin time
    5. Platelet count

    Author of lecture Hemophilia

     Carlo Raj, MD

    Carlo Raj, MD

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