Myeloproliferative Disorders Overview
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Questions and Answers

What gene mutation is associated with Polycythemia Vera?

  • EGFR mutation
  • JAK2 genetic mutation (correct)
  • BCR-ABL fusion gene
  • TP53 mutation
  • Which of the following symptoms is commonly associated with Polycythemia Vera?

  • Lymphadenopathy
  • Unexplained weight loss
  • Elevated blood pressure
  • Epistaxis (nose bleed) (correct)
  • What laboratory findings are indicative of Polycythemia Vera?

  • WBC: ↓
  • Platelets: ↓
  • H/H: ↑ (hgb > 16, Hct > 48) (correct)
  • EPO: ↑
  • Which treatment is appropriate for someone diagnosed with Polycythemia Vera?

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

    In the context of Essential Thrombocytosis, what is a common symptom?

    <p>Erythromelalgia (red hands)</p> Signup and view all the answers

    What is a known risk for patients with Essential Thrombocytosis?

    <p>Increased risk of thrombosis</p> Signup and view all the answers

    What condition can occur as a primary complication of Essential Thrombocytosis?

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

    Which test result would NOT be expected in a diagnosis of Polycythemia Vera?

    <p>Increased EPO levels</p> Signup and view all the answers

    What is the significance of the JAK2 mutation in myeloproliferative disorders?

    <p>It helps confirm the diagnosis.</p> Signup and view all the answers

    What distinguishes primary Polycythemia Vera from secondary causes of polycythemia?

    <p>Normal RBC morphology</p> Signup and view all the answers

    What is the most likely reason for elevated WBC count above 11,000?

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

    Which condition is indicated by a WBC count greater than 100,000?

    <p>Emergent evaluation</p> Signup and view all the answers

    Which factor is most associated with lymphocytosis when ALC is greater than 4000?

    <p>Viral illness</p> Signup and view all the answers

    Which test would most likely be used to confirm anemia caused by vitamin B12 deficiency?

    <p>Methylmalonic acid</p> Signup and view all the answers

    What finding in a peripheral smear indicates macrocytic anemia related to vitamin B12 deficiency?

    <p>Hypersegmented neutrophils</p> Signup and view all the answers

    Aplastic anemia primarily results from failure of what type of cells?

    <p>Hematopoietic stem cells</p> Signup and view all the answers

    Which of the following is NOT a common sign of neutropenia?

    <p>Easy bruising</p> Signup and view all the answers

    What is most likely the cause of folate deficiency in pregnant women?

    <p>Increased demand</p> Signup and view all the answers

    What is a typical characteristic finding in the peripheral smear of folate deficiency anemia?

    <p>Hypersegmented neutrophils</p> Signup and view all the answers

    What is the primary treatment recommended for aplastic anemia?

    <p>Bone marrow transplant</p> Signup and view all the answers

    The presence of immature cells in the peripheral smear is NOT characteristic of which condition?

    <p>Aplastic anemia</p> Signup and view all the answers

    Which condition can lead to increased methylmalonic acid and is associated with anemia?

    <p>Vitamin B12 deficiency</p> Signup and view all the answers

    What major lab finding is indicative of chronic hemolysis?

    <p>Elevated reticulocyte count</p> Signup and view all the answers

    In the context of drug-induced anemia, which medication is most commonly associated with macrocytic anemia?

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

    Study Notes

    Myeloproliferative Disorders

    • Myeloproliferative neoplasms are a group of blood cancers that develop because of an acquired abnormality in hematopoietic stem cells.
    • This abnormality leads to an overproduction of one or more types of blood cells.
    • Polycythemia vera is a myeloproliferative neoplasm that is characterized by an overproduction of red blood cells.
    • Risk factors include a JAK2 genetic mutation, a predisposition to the mutation, and acquired mutations after exposure.
    • S&S include headache, dizziness, tinnitus, blurred vision, fatigue, epistaxis, pruritis, conjunctival injection, engorged retinal veins, and a palpable spleen.
    • Diagnostics include elevated hemoglobin and hematocrit levels, elevated white blood cell count, elevated platelet count, normal red blood cell morphology, decreased erythropoietin levels, and elevated vitamin B12 levels.
    • Treatment includes phlebotomy to reduce blood volume, low-dose aspirin to prevent blood clots, and smoking cessation.
    • Prognosis is typically good, with a 15-year survival rate of 80%.
    • Essential Thrombocytosis is a myeloproliferative disorder that involves an overproduction of platelets.
    • Risk factors include JAK2 mutation, and being between 50 and 60 years old, women are more likely to have it than men.
    • S&S include thrombosis, erythromelalgia, and bleeding (rare).
    • Diagnostics includes elevated platelet count, elevated white blood cell count, normal red blood cell count, and a JAK2 mutation.
    • Treatment includes hydroxyurea or anagrelide to reduce platelet production, and aspirin to prevent blood clots.
    • Prognosis can range from asymptomatic to progression into myelofibrosis, leukemia, splenic infarction, or polycythemia vera.

    White Blood Cell Disorders

    • Leukocytosis is a condition in which the white blood cell count is elevated.
    • Risk factors include infection, inflammation, medications, asplenia, cigarette smoking, stress, exercise, and malignancies.
    • S&S include weight loss, fever, lymphadenopathy, rash, and drug use.
    • Diagnostics include a complete blood count, comprehensive metabolic panel, bone marrow biopsy, and flow cytometry.
    • Treatment is directed at the underlying cause.
    • Leukopenia is a condition in which the white blood cell count is low.
    • Risk factors include neutropenia and insufficient production of intrinsic factor.
    • S&S include fever, neurologic symptoms, glossitis, and signs of anemia.
    • Diagnostics include complete blood count, serum vitamin B12 level, homocysteine level, methylmalonic acid level, reticulocyte count, peripheral smear, and bone marrow biopsy
    • Treatment includes supplementation with vitamin B12 and treatment of the underlying cause.

    Macrocytic Anemia

    • Folate deficiency is a type of macrocytic anemia characterized by a lack of folate in the body.
    • Risk factors include inadequate intake, increased demand, increased loss, and medications that interfere with absorption.
    • S&S include symptoms of anemia, glossitis, and no neurologic signs,
    • Diagnostics include decreased hemoglobin, elevated MCV, decreased folate levels, and macro-ovalocytes and hypersegmented neutrophils on peripheral smear.
    • Treatment includes oral folic acid supplementation.

    Aplastic Anemia

    • Aplastic Anemia is a bone marrow failure that results in pancytopenia.
    • Risk factors include autoimmune disorders, congenital defects, viral infections, pregnancy, medications, and toxins.
    • S&S include anemia, neutropenia, thrombocytopenia, and signs of anemia.
    • Diagnostics include pancytopenia, decreased reticulocyte count, and a bone marrow biopsy that shows hypocellularity.
    • Treatment includes erythroid growth factors, myeloid growth factors, transfusions of red blood cells and platelets, and antibiotics for infection.
    • Prognosis is variable and depends on the severity of the anemia and the cause.  

    Heparin-Induced Thrombocytopenia

    • Heparin-Induced Thrombocytopenia (HIT) is a potentially life-threatening condition that occurs when a patient develops antibodies against heparin.
    • Risk factors include exposure to unfractionated heparin and having a history of HIT.
    • S&S can be asymptomatic initially, but as the condition worsens, it can cause thrombosis, bleeding, and platelet count decrease.
    • Diagnostics include PF4 Ab (ELISA), and use the 4T score to predict risk.
    • Treatment includes stopping heparin, switching to an alternative anticoagulant, and a consultation with a hematologist.

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    Heme Exam 2 Disorders PDF

    Description

    Explore the complexities of myeloproliferative neoplasms, a group of blood cancers resulting from abnormalities in hematopoietic stem cells. This quiz covers key aspects such as polycythemia vera, risk factors, symptoms and signs, diagnostics, and treatment options. Test your understanding of this critical area in hematology.

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