Beta Thalassemia: Clinical Pathology

by Carlo Raj, MD

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    00:00 Okay, let's take a look at laboratory. We'll take a look at major.

    00:03 Another name for major is called Cooley's anemia.

    00:06 There's going to be decreased hemoglobin, hematocrit, and MCV.

    00:09 The RBC count will be variant and hemoglobin electrophoresis for major is going to be what, please? No hemoglobin A, majority or hemoglobin is in form of hemoglobin F, and you'll also have hemoglobin with? Very good, A2. What must you do with this patient, you must give this patient transfusion.

    00:28 If transfusions are not given, then please understand, your patient as a child is going to die within a few months.

    00:36 However, when there is adequate perfusion -- transfusion, excuse me, transfusion, and this has been going on for 15, 20 years, secondary hemochromatosis, heart disease, two types.

    00:48 It could either be restrictive cardiomyopathy, which is, now, how do you know this? Well, they might give you restrictive cardiomyopathy.

    00:54 Now, take your time. You tell me. What kind of dysfunction is this? If you're restricting the heart, you can't feel it. You cannot.

    01:01 So, this is, good, a diastolic dysfunction.

    01:05 Or what if the iron got into the myocardial fibers and destroyed it? That's dilated cardiomyopathy, what kind of dysfunction is this? Good, systolic dysfunction. Either case, look how young your patients are, 20s.

    01:19 If not transfused, then results in heart failure. It's a high output type because it decrease.

    01:24 What does high output mean here? Severe decrease in viscosity, you're gonna have increased cardiac output, high output, but what about the tissue? You don't have any hemoglobin A so how can you possibly feed the tissues? Not gonna happen. Death in infancy.

    About the Lecture

    The lecture Beta Thalassemia: Clinical Pathology by Carlo Raj, MD is from the course Microcytic Anemia – Red Blood Cell Pathology (RBC).

    Included Quiz Questions

    1. Cardiovascular complications
    2. Splenic rupture
    3. Stroke
    4. Non-hematologic malignancies
    5. Hematologic malignacies
    1. Autosplenectomy
    2. Bony changes
    3. Stunted growth
    4. High output cardiac failure

    Author of lecture Beta Thalassemia: Clinical Pathology

     Carlo Raj, MD

    Carlo Raj, MD

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    By Audrey-Anne L. on 15. January 2018 for Beta Thalassemia: Clinical Pathology

    The videos are very clear, well explained and not too long. You can question yourself all the way along