Hematologic Disorders and Anemia Overview
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Questions and Answers

What are the three broad categories of anemia?

  • Iron Deficiency, Vitamin B12 Deficiency, Folic Acid Deficiency
  • Bleeding, Blood Deficiency, Cell Destruction
  • Loss of RBC's, Hypoproliferative Anemia, Hemolytic Anemia (correct)
  • A high speed of development in anemia typically leads to less severe symptoms.

    False

    What is the most common trigger for acute chest syndrome in children?

  • Lack of sleep
  • Dehydration
  • Infection (correct)
  • Stress
  • What is the primary function of platelets?

    <p>Clotting</p> Signup and view all the answers

    What type of anemia is most common in children?

    <p>Iron Deficiency Anemia</p> Signup and view all the answers

    What genetic disorder is characterized by the replacement of normal hemoglobin with sickle hemoglobin?

    <p>Sickle Cell Anemia</p> Signup and view all the answers

    What is the expected lifespan of a normal red blood cell?

    <p>100 days</p> Signup and view all the answers

    What is the medical emergency associated with the blockage of small vessels in the lungs?

    <p>Acute Chest Syndrome</p> Signup and view all the answers

    The primary treatment for sickle cell crisis is pain management.

    <p>False</p> Signup and view all the answers

    Which of the following is a common symptom of ITP (Immune Thrombocytopenic Purpura)?

    <p>Prolonged bleeding</p> Signup and view all the answers

    Hemophilia is a genetic bleeding disorder that primarily affects females.

    <p>False</p> Signup and view all the answers

    Which of the following clotting factors is deficient in Hemophilia B?

    <p>Factor IX</p> Signup and view all the answers

    Desmopressin (DDAVP) is an effective treatment for both Hemophilia A and Hemophilia B.

    <p>False</p> Signup and view all the answers

    What is the primary role of the von Willebrand factor?

    <p>To help platelets stick together and stabilize factor VIII</p> Signup and view all the answers

    What is the term used to describe bleeding into a joint cavity?

    <p>Hemarthrosis</p> Signup and view all the answers

    The primary goal of treatment for hemophilia is to prevent bleeding.

    <p>True</p> Signup and view all the answers

    The term 'pancytopenia' refers to a deficiency in all types of blood cells.

    <p>True</p> Signup and view all the answers

    What does the suffix '-penia' indicate?

    <p>Deficiency</p> Signup and view all the answers

    What does the prefix 'hemo-' indicate?

    <p>Relating to blood</p> Signup and view all the answers

    What does the suffix '-cyte' or '-cyto' indicate?

    <p>Cell</p> Signup and view all the answers

    The prefix 'poly-' indicates a deficiency.

    <p>False</p> Signup and view all the answers

    The prefix 'hypo-' indicates an excess.

    <p>False</p> Signup and view all the answers

    Study Notes

    Hematologic Disorders

    • Red blood cells (RBCs) or erythrocytes are responsible for transporting nutrients and oxygen to body tissues and removing waste products.
    • Platelets or thrombocytes are responsible for blood clotting.
    • White blood cells (WBCs) or leukocytes, including granulocytes (neutrophils, eosinophils, and basophils) and agranulocytes, fight infection.

    Anemia

    • Anemia is a decrease in RBCs or hemoglobin, resulting in reduced oxygen delivery to tissues.
    • Three categories of anemia:
      • Loss of RBCs (e.g., bleeding)
      • Hypoproliferative anemia (impaired RBC or hemoglobin production)
      • Hemolytic anemia (excessive RBC destruction)
    • Factors determining anemia severity:
      • Speed of development (rapid onset = more severe)
      • Duration (acute vs. chronic)
      • Client metabolic needs (more active = increased needs)
      • Concurrent disorders/disabilities

    Symptoms of Anemia

    • General symptoms: Weakness, fatigue, and malaise
    • Eyes: Yellowing
    • Respiratory system: Shortness of breath
    • Cardiovascular system: Chest pain, angina, heart attack
    • Muscular system: Weakness
    • Gastrointestinal system: Changes in stool color
    • Neurological system: Peripheral numbness, ataxia (loss of coordination), confusion
    • Skin: Pallor, jaundice, easy bruising
    • Other: Bleeding gums, angular cheilosis, tachycardia, palpations, dyspnea, dizziness, brittle/ridged/concave nails, nausea, vomiting, melena stools, anorexia, presence of coffee ground emesis, menstrual flow changes

    Types of Anemia

    • Lead poisoning: A nonessential metal; causes RBC hypo-proliferation and possibly hemolysis when present in the body.
    • Vitamin B12 deficiency: Deficiency in a critical enzyme cofactor for DNA synthesis; can cause anemia and occurs with:
      • Inadequate intake
      • Atrophic gastritis (thinning of stomach lining)
    • Folic acid deficiency: Deficiency in folic acid; can be caused by alcoholism, malabsorption, pregnancy, or lactation.
    • G6PD deficiency: An inherited enzyme deficiency that causes RBC destruction faster than production. Fava beans are a common trigger.
    • Other hemolytic anemias: Anemia caused by autoimmune disease, specific infections (e.g., malaria), or adverse medication reactions.

    Iron Deficiency Anemia

    • Most common type of anemia, especially in children. Iron is crucial for hemoglobin production.
    • Risk factors vary by age group:
      • Infants (exclusive breastfeeding beyond 6 months): Breast milk may not be a sufficient iron source.
      • Toddlers: Picky eating and excessive cow's milk consumption.
      • Adolescents: Increased dietary needs and potential poor dietary choices, menstruation.

    Expected Lab Findings

    • Complete blood count (CBC) showing low RBC, hemoglobin (Hgb), and hematocrit (Hct) values
    • RBC indices reflecting red blood cell size (MCV) and hemoglobin content per RBC (MCH)
    • Low ferritin (iron stores)
    • Low TIBC (transferrin saturation)

    Anemia Management

    • Aim to address the underlying cause of the anemia.
    • Administer iron supplements (oral or IV)
    • Manage symptoms and complications of anemia.
    • Consider blood transfusions in severe cases to boost oxygen-carrying capacity.

    Sickle Cell Anemia

    • Genetic disorder causing abnormal hemoglobin (sickle hemoglobin).
    • RBCs become rigid and sticky, leading to vessel blockage and causing pain and damage to organs.
    • Chronic condition involving various crises
    • Common amongst African Americans (1 in 365 have disease, 1 in 13 have trait)

    Sickle Cell Crises

    • Sequestration crisis: Blood pooling in the liver and spleen, causing decreased blood volume and shock.
    • Aplastic crisis: Decreased RBC production, leading to profound anemia. Triggered by viral infection or folic acid depletion.
    • Hyperhemolytic crisis: Accelerated RBC destruction characterized by anemia, jaundice, and reticulocytosis.
    • Vaso-occlusive crisis: Sickled cells block blood vessels, causing severe pain (especially in bones, joints, chest, or abdomen), inflammation, and tissue damage (necrosis).

    Clinical Manifestations of Sickle Cell Anemia

    • Central nervous system: Stroke (CVA) is a risk due to blocked blood vessels; also paralysis, retinopathy (blindness), and hemorrhage.
    • Hematologic: Hemolysis (breakdown of RBC), splenic sequestration (pooling of blood in the spleen).
    • Hepatomegaly / Splenomegaly: Enlarged liver or spleen
    • Musculoskeletal: Avascular necrosis (bone damage from reduced blood flow), dactylitis (painful swelling of hands and feet), priapism (prolonged erection), osteomyelitis.
    • Renal: Hematuria
    • Other: Jaundice, abdominal pain, fever, infection (pneumonia), fatigue and weakness, visual disturbances, shortness of breath.

    Managing Sickle Cell Anemia

    • Pain management (including opioids, on schedule), hydration, oxygen therapy
    • Education to minimize triggers (infection, dehydration, stress)
    • Treat acute crises (e.g., blood transfusions, antibiotics, hydration)
    • Prophylactic management (e.g., hydroxyurea, penicillin, vaccines)
    • Screen for complications (e.g., stroke, acute chest syndrome).

    Immune Thrombocytopenic Purpura (ITP)

    • Immune system mistakenly attacks and destroys platelets.
    • Usually develops 1-3 weeks after infection.
    • Risk factors include autoimmune disorders, certain medications, and pregnancy.
    • Signs and symptoms: Easy bruising, petechiae or purpura, prolonged or spontaneous bleeding, bleeding gums or nosebleeds, menorrhagia, internal bleeding

    Hemophilia

    • Genetic bleeding disorder: Deficiency of clotting factors (factors VIII or IX) causes excessive bleeding.

    • Types: Hemophilia A (factor VIII deficiency), Hemophilia B (factor IX deficiency), von Willebrand disease (related clotting factor).

    • Clinical Presentation: Spontaneous or prolonged bleeding, easy bruising, menorrhagia, hematochezia, hematuria, hemarthrosis (bleeding into joints), intracranial bleeding.

    • Complications: Severe or recurrent hemarthrosis, permanent joint deformity, disability.

    • Lab findings: Prolonged aPTT, factor VIII/IX levels low in Hemophilia A/B, normal PT.

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    Description

    This quiz covers key concepts related to hematologic disorders, focusing on the structure and function of red blood cells, platelets, and white blood cells. It also delves into anemia, including its types, symptoms, and factors affecting severity. Test your understanding of these critical aspects of hematology.

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