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Pyruvate Kinase Deficiency: Morphology and Clinical Pathology

by Carlo Raj, MD
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    00:00 So what then happens to your RBC? It gets spiculated.

    00:06 In other words, your RBC looks like it has little needle projections from the RBC.

    00:12 So you definitely know that the RBC is misshapen.

    00:14 And most likely, something to do with the decreased production of ATP because you’re stuck at? Well, you’re stuck at phosphoenolpyruvate, right? And whenever you have a misshapen RBC, where does it go for destruction? Hereditary spherocytosis, hereditary elliptocytosis, sickle cell.

    00:34 All of them are going to go to the spleen, resulting in? Extravascular hemolysis.

    00:38 Now, this is a child.

    00:40 Whenever you have an enzyme deficiency, you’re born with a disorder.

    00:45 Serum methemoglobin is elevated.

    00:47 Why? NADH is inadequate.

    00:53 Almost, well, most common error in glycolysis.

    00:56 However, in terms of a disease though, it’s quite rare, but make sure that you know in greater detail all of the different points that we made here about pyruvate kinase deficiency.

    01:07 What are you going to find? This RBC is going to take on bizarre shape.

    01:11 And this bizarre shape is then known as an echinocyte, most likely due to as we said, inadequate amounts of ATP, having some type of influence in a negative manner on the sodium potassium pump in which you end up finding here on a peripheral blood smear are these weird spiculated type of echinocytes.


    About the Lecture

    The lecture Pyruvate Kinase Deficiency: Morphology and Clinical Pathology by Carlo Raj, MD is from the course Hemolytic Anemia – Red Blood Cell Pathology (RBC).


    Included Quiz Questions

    1. Phosphoenolpyruvate
    2. Pyruvate carboxylase
    3. Pyruvate dehydrogenase
    4. Phosphoglycerate kinase
    5. Pyruvate kinase
    1. Increased DPG
    2. Decreased PCO2
    3. Decreased hydrogen ions
    4. Increased pH
    5. Decreased temperature
    1. Methemoglobin
    2. Anemia
    3. Fever
    4. Pyruvate kinase deficiency
    5. Acute acidosis
    1. Echinocyte
    2. Enterocyte
    3. Pinacocyte
    4. Solenocyte
    5. Electrocyte

    Author of lecture Pyruvate Kinase Deficiency: Morphology and Clinical Pathology

     Carlo Raj, MD

    Carlo Raj, MD


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