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Hemolytic Anemia: Hereditary Spherocytosis (HS) – Red Blood Cell Pathology

by Carlo Raj, MD
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    Let’s take a look at hereditary spherocytosis. HS is what I’m going to refer to this as. Hereditary spherocytosis is a membrane issue and the name pretty much tells you everything that you need to know. Where am I in terms of your category? You are normocytic anemia, you’re looking at MCV between 80 to 100. So what then happens when there’s enough membrane loss? Now, in your mind, conceptually work with me here. And a normal RBC is biconcave, whatever that means. And point being is it has central pallor, right? And so therefore, if you start losing parts of the membrane, what then happens? The membrane is then going to become more and more of a spherocyte. It loses its "biconcave shape." What then happens to the central pallor? It’s no longer present. So it’s highly concentrated. Now you give me another test in which it then tells you that you have a highly concentrated RBC, where the RBCs homogenously reddened or are hyperchromic. MCHC. Clear? So we have two major RBC index that we’ll take a look at. Number one, it’s a fact that, well, your normocytic is where we are, 80 to 100. Number two, in terms of reticulocytosis, you expect your reticulocyte production index to be increased or decreased here, please? Increased. And we find MCHC because of increased hemoglobin concentration to be high as well. So what is this important membrane component that’s missing in HS? Spectrin, spectrin, spectrin. You also have ankyrin as well, but spectrin is a major, major membrane component. Now, those of you that are interested in going into hematology, you’ll be dealing with and spending a lot of time with that RBC membrane for various reasons. There are all of these antigens. You all know about D antigen, right?...

    About the Lecture

    The lecture Hemolytic Anemia: Hereditary Spherocytosis (HS) – Red Blood Cell Pathology by Carlo Raj, MD is from the course Hemolytic Anemia – Red Blood Cell Pathology (RBC). It contains the following chapters:

    • Hereditary Spherocytosis - Etiology
    • Hereditary Spherocytosis - Pathogenesis
    • Hereditary Spherocytosis - Clinical Pathology

    Included Quiz Questions

    1. Spectrin
    2. Globin
    3. Hepcidin
    4. Haptoglobin
    5. Transferrin
    1. Loss of central pallor
    2. None of these
    3. Increased surface area
    4. Biconcave shape
    5. Hypochromic
    1. Autoimmune hemolytic anemia (warm type)
    2. Autoimmune hemolytic anemia (cold type)
    3. Sickle cell anemia
    4. Beta thalassemia
    5. Paroxysmal nocturnal hemoglobinuria
    1. Negative coombs test in spherocytosis
    2. Negative coombs test in autoimmune hemolytic anemia
    3. Negative osmotic fragility test in spherocytosis
    4. Positive osmotic fragility test in autoimmune hemolytic anemia
    5. Positive coombs test in spherocytosis
    1. Osmotic fragility test
    2. Coombs test
    3. Metabisulfate screen
    4. Acidified serum test
    5. Sucrose test
    1. Damage to Na/K ATPase pump in the membrane
    2. Increased surface area of RBC
    3. Damage to Na/Ca pump in the membrane
    4. Damage to Na/Glucose pump in the membrane
    5. Decreased mean hemoglobin concentration
    1. Aplastic crisis
    2. Increased hemolysis
    3. Production of antibodies against RBCs
    4. Increased RBC life span
    5. Decreased hemolysis

    Author of lecture Hemolytic Anemia: Hereditary Spherocytosis (HS) – Red Blood Cell Pathology

     Carlo Raj, MD

    Carlo Raj, MD


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