Medicine Marrow Pg 101-110 (Hematology)
50 Questions
0 Views

Medicine Marrow Pg 101-110 (Hematology)

Created by
@ArdentHouston

Podcast Beta

Play an AI-generated podcast conversation about this lesson

Questions and Answers

Which of the following conditions is NOT a cause of secondary polycythemia?

  • Chronic obstructive pulmonary disease (COPD)
  • Renal artery stenosis
  • Polycythemia vera (correct)
  • High altitude

    • In primary polycythemia, erythropoietin levels are typically increased.

    False

    What is the recommended daily hydration amount for patients with no symptoms of myeloproliferative neoplasms?

    3-4 liters

    In secondary polycythemia due to renal artery stenosis, erythropoietin levels are typically __________.

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

    Match the conditions to their associated characteristics:

    <p>COPD = Hypoxia related secondary polycythemia Pheochromocytoma = Paraneoplastic secondary polycythemia Cerebellar hemangioblastoma = Associated with von Hippel-Lindau syndrome Uterine fibroids = Paraneoplastic condition that can cause polycythemia</p> Signup and view all the answers

    What is a key characteristic of Childhood Hemolytic Uremic Syndrome (HUS)?

    <p>Triggered by bacterial infections</p> Signup and view all the answers

    All cases of Hemolytic Uremic Syndrome are related to infections.

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

    What is the primary organ affected by Hemolytic Uremic Syndrome?

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

    HUS often results in a deficiency of __________ due to the destruction of red blood cells.

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

    Match the following characteristics with the correct type of HUS:

    <p>Childhood HUS = Often preceded by gastroenteritis Atypical HUS = Associated with genetic mutations Typical HUS = Commonly caused by E. coli Thrombotic Thrombocytopenic Purpura (TTP) = Adults mostly affected</p> Signup and view all the answers

    Which of the following is an example of an enzyme deficiency associated with inherited hemolytic anemia?

    <p>G6PD deficiency</p> Signup and view all the answers

    Hereditary spherocytosis is an autosomal recessive condition.

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

    What is the most abundant protein on the red blood cell (RBC) membrane that Plasmodium falciparum binds to?

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

    The most common enzyme deficiency seen in inherited hemolytic anemia is __________.

    <p>G6PD deficiency</p> Signup and view all the answers

    Match the following diseases with their associated characteristics:

    <p>Hereditary spherocytosis = Defective pattern of lipid bilayer Sickle cell anemia = Hemoglobinopathy Thalassemia = Genetic disorder affecting hemoglobin production Hereditary ovalocytosis = Associated with cytoskeletal defects</p> Signup and view all the answers

    What is the primary cause of Thrombotic Thrombocytopenic Purpura (TTP) in most cases?

    <p>Idiopathic autoantibodies against ADAMTS-13</p> Signup and view all the answers

    The platelet count in microangiopathic hemolytic anemia (MAHA) is usually normal.

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

    What is the main therapeutic approach for managing Thrombotic Thrombocytopenic Purpura (TTP)?

    <p>Plasmapheresis, steroid, and rituximab</p> Signup and view all the answers

    In TTP, autoantibodies against ADAMTS-13 lead to the accumulation of _____ in circulation.

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

    Match the following causes of hemolysis with their associated platelet counts:

    <p>MAHA/march hemolysis = Low Cardiac related hemolysis = Normal Consumption = Low</p> Signup and view all the answers

    What is a common treatment for moderate hemolytic anemia in adults?

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

    Splenectomy is indicated for severe disease in children as early as 3-4 years.

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

    What is the main enzyme deficient in G6PD deficiency?

    <p>Glucose 6-phosphate dehydrogenase</p> Signup and view all the answers

    One of the precipitating factors for G6PD deficiency is __________.

    <p>fava beans</p> Signup and view all the answers

    Match the precipitating drugs with their potential effects on G6PD deficiency:

    <p>Primaquine = Acute intravascular hemolysis Sulfamethoxazole = Acute intravascular hemolysis High-dose aspirin = Risk of hemolytic episode Chloroquine = Acute intravascular hemolysis</p> Signup and view all the answers

    Which mutation is most commonly associated with Polycythemia rubra vera (PCRV)?

    <p>JAK-2 mutation</p> Signup and view all the answers

    Primary myelofibrosis is classified under myeloproliferative neoplasms.

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

    Name one terminal myeloid cell type.

    <p>RBC, platelets, neutrophils, monocytes, eosinophils, or basophils</p> Signup and view all the answers

    Chronic myeloid leukemia (CML) is characterized by the __________ mutation.

    <p>BCR-ABL translocation</p> Signup and view all the answers

    Match the following myeloproliferative neoplasms with their associated mutations:

    <p>Polycythemia rubra vera = JAK-2 mutation Chronic myeloid leukemia = BCR-ABL translocation Chronic eosinophilic leukemia = PDGFRA mutation Chronic neutrophilic leukemia = CSF3R mutation</p> Signup and view all the answers

    What is the significance of Howell-Jolly bodies in a peripheral smear?

    <p>They are indicative of splenic dysfunction</p> Signup and view all the answers

    Bite cells are formed due to the degradation of hemoglobin in red blood cells.

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

    What are the three stages of blood loss in relation to anemia?

    <p>Hypovolemia, Volume shift, EPO ↑ and reticulocyte ↑</p> Signup and view all the answers

    The three components of the triad associated with severe anemia due to blood loss are __________, hypothermia, and DIC.

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

    Match the following types of red blood cell inclusions with their description:

    <p>Howell-Jolly bodies = Small, dark spherical inclusions within RBCs Heinz bodies = Inclusions that indicate denatured globin proteins Bite cells = Cells with irregular bite-like shapes Blister cells = RBCs with irregular shaped membrane blebs</p> Signup and view all the answers

    What is the most common mutation found in patients with Primary Polycythemia?

    <p>Exon 14 mutation V617F</p> Signup and view all the answers

    Moderate splenomegaly is a clinical feature of Primary Polycythemia.

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

    What is a common symptom experienced due to hyperviscosity in Primary Polycythemia?

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

    In Primary Polycythemia, patients often experience __________, which is burning pain of the hands and feet.

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

    Match the clinical features with their descriptions related to Primary Polycythemia:

    <p>Erythrocytosis = Increased red blood cell mass Thrombocytosis = Increased platelet count leading to bleeding Basophilia = Pruritus after bathing Hyperuricemia = High uric acid levels due to cell turnover</p> Signup and view all the answers

    What is the main consequence of the defect in the lipid bilayer of red blood cells in this condition?

    <p>Formation of microvesicles</p> Signup and view all the answers

    Anemia is a clinical feature associated with this condition.

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

    What is the most preferred method for evaluating red blood cell deformability?

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

    RBCs typically have a diameter of ________ µm.

    <p>7-8</p> Signup and view all the answers

    Match the investigations with their key features:

    <p>Peripheral smear = Microspherocytes present SDS page electrophoresis = Gold standard investigation Osmotic fragility test = RBC ruptures at lower saline concentration Eosin 5 maleimide (EMA) test = Fluorescence binding to RBCs</p> Signup and view all the answers

    Which of the following conditions is associated with moderate extramedullary hematopoiesis?

    <p>Polycythemia Vera (PCV)</p> Signup and view all the answers

    Hyperviscosity symptoms include headache, vertigo, and tinnitus.

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

    What is the primary cause of pancytopenia in Myelodysplastic Syndrome (MDS)?

    <p>Ineffective proliferation of terminal myeloid cells</p> Signup and view all the answers

    Dehydration leads to __________ relative polycythemia.

    <p>Gaisbock's syndrome</p> Signup and view all the answers

    Match the types of polycythemia with their descriptions:

    <p>Relative Polycythemia = Decreased plasma volume leading to increased Hb Absolute Polycythemia = Increased red blood cell mass leading to elevated Hb Polycythemia Vera = A type of myeloproliferative neoplasm Secondary Polycythemia = Increased erythropoietin production due to hypoxia</p> Signup and view all the answers

    Study Notes

    Myeloproliferative Neoplasms (MPNs)

    • MPNs are characterized by slow proliferation of mature myeloid cells from multiple lineages, without presence of immature cells.
    • The WHO 5th edition classifies eight MPNs based on phenotypic heterogeneity and mutations.
    • Polycythemia rubra vera (PCRV), the most common MPN, is characterized by JAK2 mutation, primarily seen in exon 14 as V617F mutation.
    • Other MPNs include primary myelofibrosis (PMF), essential thrombocytosis/thrombocythemia (ET), chronic myeloid leukemia (CML), chronic neutrophilic leukemia (CNL), chronic eosinophilic leukemia (CEL), juvenile myelomonocytic leukemia (JMML) and MPN, not otherwise specified.

    Secondary Polycythemia

    • Secondary polycythemia develops due to increased EPO production due to underlying conditions like hypoxia, renal artery stenosis, and paraneoplastic syndromes.
    • Hypoxia can be caused by various conditions including COPD, OSAS, high altitude, CO intoxication, and hepatopulmonary syndrome.
    • Paraneoplastic syndromes include renal carcinoma, cerebellar hemangioblastoma associated with von Hippel-Lindau syndrome, meningioma, pheochromocytoma, uterine fibroids, and hepatoma.

    Hemolytic Uremic Syndrome (HUS)

    • HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury.
    • It can be classified into childhood and adult types.
    • The most common cause of adult HUS is IgG autoantibodies against ADAMTS13, a protein responsible for cleaving von Willebrand factor (vWF).
    • HUS is often a life-threatening condition, requiring prompt diagnosis and treatment, including plasmapheresis, steroids, and rituximab.

    Hemolytic Anemia: Miscellaneous

    • Inherited hemolytic anemias can be categorized into hemoglobinopathies, membrane cytoskeleton disorders, and enzymopathies.
    • Hemoglobinopathies include sickle cell anemia and thalassemia.
    • Membrane cytoskeleton disorders include hereditary spherocytosis, a condition with autosomal dominant inheritance pattern caused by defects in ankyrin, Band 3, or B-spectrin.
    • Enzymopathies involve deficiencies in G6PD, pyruvate kinase, and S' nucleotidase.
    • S' nucleotidase deficiency is characterized by basophil stippling and markers of cholestasis.

    Hereditary (Congenital) Spherocytosis (HS)

    • HS is a chronic compensated extravascular hemolytic anemia caused by a defect in RBC membrane lipid bilayer, affecting vertical and tangential associations.
    • This leads to microvesical formation, detachment, and spherocytosis, causing increased osmotic fragility of RBCs.

    Thrombotic Thrombocytopenic Purpura (TTP)

    • TTP is characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and thrombosis in small blood vessels.
    • It's primarily caused by autoantibodies against ADAMTS-13, leading to accumulation of uncleaved vWF, causing platelet aggregation and microthrombi formation.

    Blood Loss

    • Blood loss is a common cause of anemia, triggering different stages of physiological response.
    • Initial stages involve hypovolemia, followed by volume shift from extravascular to intravascular space.
    • This process, involving EPO and reticulocyte production, can potentially lead to a triad of acidosis, hypothermia, and DIC (Disseminated Intravascular Coagulation).

    Polycythemia

    • Polycythemia refers to an increase in red blood cell count, categorized as relative or absolute.
    • Relative polycythemia, also termed Gaisbock's syndrome, occurs due to decreased plasma volume, often associated with dehydration or post-viral infection like Dengue.
    • Absolute polycythemia is further classified into primary (PCRV) and secondary.
    • Primary polycythemia (PCRV), also known as Polycythemia vera, is a myeloproliferative neoplasm caused by JAK2 mutation and characterized by increased RBC mass, granulocytosis, thrombocytosis, and splenomegaly.

    G6PD Deficiency

    • G6PD deficiency is an X-linked intermediate deficiency that causes an inability to produce adequate NADPH, crucial for reducing oxidative stress in RBCs.
    • It leads to acute intravascular hemolysis and chronic extravascular hemolysis.
    • Precipitating factors include certain drugs, infections, and consumption of fava beans.
    • Heterozygous carriers of G6PD deficiency exhibit resistance to malaria.

    Treatment

    • Treatment for G6PD deficiency primarily involves avoiding precipitating drugs, infections, and fava beans.
    • For severe cases, supportive care, splenectomy, and management of complications such as jaundice and gallstones may be needed.

    Blood Transfusion

    • Blood transfusions are often used to address anemia caused by various conditions including blood loss, hemolytic anemia, and myelodysplastic syndromes.

    Splenectomy

    • Splenectomy is a surgical procedure involving the removal of the spleen.
    • In cases of hereditary spherocytosis, splenectomy is often performed to reduce the rate of red blood cell destruction.
    • Splenectomy is also sometimes performed for other conditions like thalassemia, sickle cell anemia, and certain types of lymphoma.

    Investigative Procedures

    • Peripheral smear analysis is a key tool for diagnosing anemias and other blood disorders.
    • It allows for visual examination of erythrocytes, leukocytes, and platelets, revealing abnormalities like Howell-Jolly bodies, Heinz bodies, spherocytes, target cells, and other morphological changes.
    • Various other test methods are used to investigate specific types of anemia, including osmotic fragility test, Ektacytometry, SDS page electrophoresis, EMA test, and quantitative assays.
    • Fluorescent spot test is a screening technique used to detect G6PD deficiency.

    Studying That Suits You

    Use AI to generate personalized quizzes and flashcards to suit your learning preferences.

    Quiz Team

    Description

    This quiz covers the essential aspects of Myeloproliferative Neoplasms (MPNs) including their classification, characteristics, and specific types. It also discusses secondary polycythemia and its underlying causes related to increased EPO production. Test your understanding of these hematological conditions and their implications.

    More Like This

    Myeloproliferative Neoplasms
    32 questions

    Myeloproliferative Neoplasms

    ComfortableGalaxy avatar
    ComfortableGalaxy
    Cytogenetics in Myeloproliferative Neoplasms
    24 questions

    Cytogenetics in Myeloproliferative Neoplasms

    Quizgecko avatar
    Quizgecko
    Myeloproliferative Neoplasms Overview
    29 questions

    Myeloproliferative Neoplasms Overview

    PlayfulFauvism7346 avatar
    PlayfulFauvism7346
    Primary Myelofibrosis Overview
    37 questions

    Primary Myelofibrosis Overview

    RosyDiction avatar
    RosyDiction
    Use Quizgecko on...
    Browser
    Open
    Browser
    Browser