Anemia and Iron Deficiency Quiz from NJN
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Questions and Answers

What is the primary defect in hereditary spherocytosis that leads to hemolytic anemia?

  • Defect in red blood cell membrane proteins, causing spherically shaped and fragile red blood cells. (correct)
  • Increased production of red blood cells leading to premature destruction.
  • Deficiency of glucose-6-phosphate dehydrogenase (G6PD).
  • Abnormal hemoglobin structure causing sickle-shaped red blood cells.

    • Which enzyme deficiency is directly associated with oxidative damage and red blood cell destruction in hemolytic anemia?

  • Glucose-6-phosphate dehydrogenase (G6PD). (correct)
  • Catalase.
  • Pyruvate kinase.
  • Superoxide dismutase.

    • Why are patients who undergo splenectomy at an increased risk of infection?

  • The spleen produces antibodies against viral infections, and its removal compromises immunity.
  • The spleen is responsible for producing red blood cells, and its removal leads to anemia.
  • The spleen filters the blood and plays a role in the immune response against encapsulated bacteria. (correct)
  • The spleen regulates blood clotting factors, and its removal leads to an increased risk of bleeding.

    • Which of the following is a reason why individuals with sickle cell anemia exhibit resistance to malaria?

    <p>Sickle cells are less susceptible to invasion by the <em>Plasmodium falciparum</em> parasite. (B)</p> Signup and view all the answers

    Following a splenectomy, which preventative measures are most critical for patients to avoid serious infections?

    <p>Vaccination against encapsulated bacteria and prophylactic antibiotics. (A)</p> Signup and view all the answers

    Which of the following laboratory findings is most indicative of iron deficiency anemia?

    <p>Decreased mean corpuscular volume (MCV) (D)</p> Signup and view all the answers

    Which of the following proteins is directly involved in maintaining the biconcave shape of red blood cells?

    <p>Spectrin (A)</p> Signup and view all the answers

    What is the primary mechanism by which antibodies cause pernicious anemia?

    <p>Binding to intrinsic factor, preventing vitamin B12 absorption (D)</p> Signup and view all the answers

    Which of the following conditions is often associated with splenomegaly due to increased red blood cell destruction?

    <p>Hereditary spherocytosis (C)</p> Signup and view all the answers

    What is the direct role of vitamin B12 in red blood cell production?

    <p>DNA maturation and condensation in red blood cells (D)</p> Signup and view all the answers

    A patient presents with fatigue, shortness of breath, and an elevated MCV. Which deficiency is most likely?

    <p>Vitamin B12 (C)</p> Signup and view all the answers

    A patient's blood test reveals a hematocrit of 35%. What condition does this suggest?

    <p>Anemia (C)</p> Signup and view all the answers

    Which enzyme, when deficient, is associated with the pentose phosphate pathway and can lead to anemia?

    <p>Glucose-6-Phosphate Dehydrogenase (B)</p> Signup and view all the answers

    Which of the following is the direct consequence of the missense mutation in sickle cell anemia?

    <p>Substitution of glutamic acid with valine in the beta chain of hemoglobin. (B)</p> Signup and view all the answers

    What is the underlying mechanism by which sickle cell anemia leads to vaso-occlusive crisis?

    <p>Polymerization of hemoglobin, leading to rigid, sickle-shaped red blood cells that obstruct capillaries. (C)</p> Signup and view all the answers

    Why is Streptococcus pneumoniae a significant concern following splenectomy in patients with sickle cell anemia?

    <p>The spleen enhances the immune response to encapsulated bacteria like <em>Streptococcus pneumoniae</em>. (A)</p> Signup and view all the answers

    How does hydroxyurea improve oxygen delivery in patients with sickle cell anemia?

    <p>By increasing the production of fetal hemoglobin, which has a higher affinity for oxygen. (D)</p> Signup and view all the answers

    What is the primary mechanism behind hemorrhagic anemia?

    <p>Reduced oxygen-carrying capacity due to blood loss. (D)</p> Signup and view all the answers

    What is a key characteristic of aplastic anemia?

    <p>Pancytopenia, a deficiency of all blood cell types. (B)</p> Signup and view all the answers

    Which of the following is a symptom directly related to thrombocytopenia in aplastic anemia?

    <p>Easy bruising and petechiae. (A)</p> Signup and view all the answers

    What is the main underlying cause of Thalassemia?

    <p>Genetic mutations leading to reduced or absent production of hemoglobin. (D)</p> Signup and view all the answers

    If a patient presents with microcytic anemia (MCV < 90 fL), which type of anemia is most likely?

    <p>Iron Deficiency (B)</p> Signup and view all the answers

    What is the mechanism by which vitamin B12 deficiency leads to anemia?

    <p>Defective DNA synthesis and cell division. (C)</p> Signup and view all the answers

    What is the effect of ROS on red blood cells in G6PD deficiency?

    <p>Damage to hemoglobin, causing it to precipitate into Heinz bodies. (C)</p> Signup and view all the answers

    What is the purpose of a Coombs test?

    <p>To detect hereditary spherocytosis. (C)</p> Signup and view all the answers

    During internal respiration, what change in the hemoglobin molecule leads to red blood cell sickling in sickle cell anemia?

    <p>Change in three-dimensional shape as oxygen leaves the molecule. (B)</p> Signup and view all the answers

    What is the direct cause of priapism in the context of vaso-occlusive crisis in sickle cell anemia?

    <p>Blocked penile arterioles by sickle-shaped red blood cells. (C)</p> Signup and view all the answers

    Which treatment is NOT typically used for hemorrhagic anemia?

    <p>Bone marrow transplantation. (C)</p> Signup and view all the answers

    Flashcards

    Anemia

    A condition with reduced oxygen-carrying capacity in the blood due to low or dysfunctional red blood cells.

    Hematocrit Test

    A blood test used to diagnose anemia by measuring the proportion of red blood cells in blood.

    Iron Deficiency Anemia

    Anemia caused by insufficient iron, leading to low hemoglobin and microcytic red blood cells.

    Microcytosis

    A condition characterized by smaller than normal red blood cells due to low iron production.

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    Pernicious Anemia

    An autoimmune disorder affecting vitamin B12 absorption, leading to large red blood cells.

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    Macrocytosis

    A condition where red blood cells are larger than normal due to B12 or folic acid deficiency.

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    Hereditary Spherocytosis

    A genetic disorder causing spherical red blood cells due to protein mutations, leading to hemolysis.

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    G6PD Deficiency

    A genetic condition caused by lack of glucose-6-phosphate dehydrogenase, affecting red blood cell metabolism.

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    Splenectomy

    A surgical procedure to remove the spleen, often needed in certain blood disorders.

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    Risk of Encapsulated Bacteria

    Patients without a spleen are at higher risk for infections from specific encapsulated bacteria.

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    Malaria Resistance

    People with sickle cell anemia are less susceptible to malaria infection due to abnormal red blood cells.

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    Pentose Phosphate Pathway

    A metabolic pathway that produces NADPH for reducing ROS and maintaining red blood cell integrity.

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    Heinz Bodies

    Aggregates of denatured hemoglobin in red blood cells due to oxidative damage.

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    Sickle Cell Anemia

    A genetic disorder causing missense mutation in hemoglobin, leading to sickle-shaped red blood cells.

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    Polymerization of Hemoglobin

    Process where hemoglobin molecules stick together, causing red blood cell deformity in sickle cell anemia.

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    Vaso-Occlusive Crisis

    A painful event when sickled red blood cells block blood flow in capillaries.

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    Priapism

    A painful, prolonged erection resulting from blocked blood flow in the penis.

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    Aplastic Anemia

    A condition marked by a deficiency of all blood cell types due to bone marrow damage.

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    Thalassemia

    A genetic disorder that causes reduced hemoglobin production due to defective globin chains.

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    Vitamin B12 Deficiency Anemia

    A macrocytic anemia caused by a lack of vitamin B12, impairing DNA synthesis.

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    Fluids in Sickle Cell Treatment

    Intravenous fluids used to restore blood volume and prevent dehydration in sickle cell patients.

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    Hydroxyurea

    A medication that stimulates the production of fetal hemoglobin to help improve oxygen delivery.

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    Blood Transfusions

    A treatment method used to increase red blood cells and improve oxygen-carrying capacity in anemia.

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    Study Notes

    Anemia

    • Anemia is characterized by a reduced oxygen-carrying capacity in the blood, often due to a low number of red blood cells or dysfunctional red blood cells.
    • A hematocrit test, measuring PCV or HCV, can diagnose anemia. Low values (less than 45%) indicate anemia.

    Iron Deficiency Anemia

    • Symptoms include dyspnea, fatigue, tachycardia, and dizziness.
    • Iron is essential for hemoglobin synthesis, responsible for oxygen transport.
    • Iron deficiency reduces heme production, leading to lower functional hemoglobin levels.
    • This results in microcytic red blood cells, characterized by a mean corpuscular volume (MCV) below 90 femtoliters.
    • Common causes include blood loss (e.g., ulcers), menorrhagia, and insufficient dietary iron intake.
    • Treatment involves iron supplementation.

    Pernicious Anemia and Folic Acid Deficiency

    • Pernicious anemia is an autoimmune condition affecting vitamin B12 absorption.
    • Parietal cells secrete intrinsic factor, vital for B12 absorption.
    • Antibodies interfere with intrinsic factor, preventing B12 absorption.
    • B12 is essential for red blood cell DNA maturation and condensation.
    • Folic acid deficiency also affects DNA maturation.
    • Insufficient B12 results in macrocytic red blood cells, characterized by an MCV above 90 femtoliters.
    • Treatment involves intramuscular B12 injections.

    Hereditary Spherocytosis

    • This genetic condition involves mutations in spectrin or ankyrin proteins, crucial for maintaining the biconcave red blood cell shape.
    • Mutations cause spherical red blood cells (spherocytosis), reducing cell flexibility.
    • Spherocytes can get trapped in capillaries, leading to hemolysis (red blood cell destruction) and splenomegaly (enlarged spleen).
    • MCV may fluctuate but is often considered microcytic.

    G6PD Deficiency

    • G6PD (glucose-6-phosphate dehydrogenase) is an enzyme in the pentose phosphate pathway.
    • The pathway produces NADPH, crucial for reducing reactive oxygen species (ROS) and red blood cell integrity.
    • G6PD deficiency reduces NADPH production, increasing ROS levels.
    • ROS damage hemoglobin, leading to Heinz body formation, decreased red blood cell flexibility, and hemolysis.

    Sickle Cell Anemia

    • This genetic disorder involves a missense mutation in the beta chain of hemoglobin.
    • The glutamic acid (GLU) is replaced with valine, altering hemoglobin structure and causing polymerization.
    • Polymerized hemoglobin distorts red blood cells into a sickle shape.
    • Sickle cells are less flexible, blocking capillaries, causing pain and organ damage.

    Hemoglobin Structure and Sickle Cell Anemia

    • The missense mutation causes hemoglobin to polymerize and sickle when not bound to oxygen.
    • Sickling alters the red blood cell's shape, making it less flexible and causing capillary blockage.
    • During internal respiration, the loss of oxygen from hemoglobin leads to sickling.
    • Rebinding of oxygen to hemoglobin reverses the sickling process.

    Vaso-occlusive Crisis and Complications of Sickle Cell Anemia

    • Vaso-occlusive crisis occurs when sickle cells block capillaries, causing complications such as:
    • Priapism: Prolonged, painful erection.
    • Splenomegaly: Enlarged spleen, potentially requiring splenectomy.
    • Splenectomy removes the spleen, increasing the risk of encapsulated bacterial infections (Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae).

    Treatment Options for Sickle Cell Anemia

    • Oxygen therapy increases blood oxygen levels, reducing sickling.
    • Pain relievers manage vaso-occlusive crises (opioids).
    • IV fluids restore blood volume, preventing dehydration.
    • Hydroxyurea increases fetal hemoglobin production, improving oxygen delivery.

    Hemorrhagic Anemia

    • Hemorrhagic anemia results from blood loss, decreasing red blood cells and oxygen-carrying capacity.
    • Causes include trauma, aneurysm rupture, or gastrointestinal bleeding.
    • Treatment often involves blood transfusions, fluids, and surgical intervention to stop bleeding.

    Aplastic Anemia

    • Aplastic anemia is a deficiency of all blood cell types: red blood cells (anemia), white blood cells (leukopenia), and platelets (thrombocytopenia).
    • Pancytopenia is often due to bone marrow damage (e.g., myeloid stem cells).
    • About 65% of cases are idiopathic, but it can be triggered by drugs (chloramphenicol, benzene, streptomycin), viruses (cytomegalovirus, Epstein-Barr virus), or radiation.

    Symptoms and Treatment of Aplastic Anemia

    • Symptoms include: anemia (fatigue, weakness), leukopenia (increased infection risk), and thrombocytopenia (easy bruising, bleeding).
    • Treatment includes bone marrow transplantation, blood transfusions, and supportive care (antibiotics, platelet transfusions).

    Thalassemia

    • Thalassemia involves reduced or absent hemoglobin production due to faulty or missing globin chains.
    • Types include alpha-thalassemia (reduced alpha globin) and beta-thalassemia (reduced beta globin).
    • The genetic mutation leads to microcytic anemia (smaller red blood cells) with an MCV below 90 femtoliters.

    Treatment of Thalassemia

    • Treatment often involves regular blood transfusions to maintain hemoglobin levels.
    • Iron supplements might be necessary due to frequent transfusions.
    • Bone marrow transplantation (ideal treatment) is not always feasible.

    Other Forms of Anemia

    • Iron deficiency anemia is a microcytic anemia due to insufficient iron stores.
    • Vitamin B12 and folic acid deficiency anemias are macrocytic, affecting DNA synthesis and cell division.

    Hemolytic Anemia

    • Hereditary spherocytosis is a genetic red blood cell membrane protein defect causing spherically-shaped, fragile red blood cells.
    • G6PDH deficiency reduces the enzyme G6PD, causing oxidative damage and red blood cell destruction.

    Additional Notes

    • Splenectomy, the surgical removal of the spleen, is sometimes needed for persistent vaso-occlusive crises in sickle cell anemia or hereditary spherocytosis, raising the risk of encapsulated bacterial infections.
    • People with sickle cell anemia have resistance to malaria due to reduced susceptibility to Plasmodium falciparum infection.

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    Description

    Test your knowledge on anemia and its types, specifically focusing on iron deficiency anemia. This quiz covers symptoms, causes, and diagnostic tests related to anemia. Learn about the importance of iron and its role in hemoglobin synthesis.

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