HLS Pathology Lecture 4: Initial Laboratory Tests for Bleeding Abnormalities
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Questions and Answers

Which of the following is the characteristic pathologic feature of Thrombotic Thrombocytopenic Purpura (TTP)?

  • Hyaline (platelet rich) microthrombi (correct)
  • Microangiopathic hemolytic anemia
  • Normocytic normochromic anemia
  • Schistocytes

    • Which laboratory finding is common in both TTP and Hemolytic Uremic Syndrome (HUS)?

  • Increase in indirect bilirubin
  • Elevated bleeding time (correct)
  • Increase in LDH
  • Decrease in haptoglobin

    • Which of the following is the primary treatment for Thrombotic Thrombocytopenic Purpura (TTP)?

  • Antihypertensive medications
  • Dialysis
  • Conservative management
  • Plasma exchange (correct)

    • Which of the following is the most common age group affected by Thrombotic Thrombocytopenic Purpura (TTP)?

    <p>3rd-4th decade</p> Signup and view all the answers

    Which of the following is the characteristic pathologic feature of Hemolytic Uremic Syndrome (HUS)?

    <p>Platelet microaggregates (hyaline microthrombi) in the glomerular capillaries</p> Signup and view all the answers

    Which of the following is the most common causative agent in Hemolytic Uremic Syndrome (HUS)?

    <p>Escherichia coli O157:H7</p> Signup and view all the answers

    Which of the following is the typical clinical presentation of Hemolytic Uremic Syndrome (HUS)?

    <p>Thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure</p> Signup and view all the answers

    Which of the following is the most appropriate treatment for Hemolytic Uremic Syndrome (HUS)?

    <p>Conservative management (dialysis, antihypertensive, etc.)</p> Signup and view all the answers

    Which of the following is a common laboratory finding in both Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS)?

    <p>Microangiopathic hemolytic anemia</p> Signup and view all the answers

    Which of the following is the key difference between Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS)?

    <p>The age group affected</p> Signup and view all the answers

    What is the most common bleeding site in patients with hemophilia A?

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

    Which of the following is a characteristic feature of hemophilia A?

    <p>Bleeding episodes are mild, moderate or severe depending on the factor VIII level</p> Signup and view all the answers

    What is the role of von Willebrand factor in hemophilia A?

    <p>Von Willebrand factor circulates bound to factor VIII and stabilizes it</p> Signup and view all the answers

    What is the typical laboratory finding in hemophilia A?

    <p>Prolonged activated partial thromboplastin time (aPTT)</p> Signup and view all the answers

    What is the most appropriate treatment for hemophilia A?

    <p>Replacement therapy with factor VIII concentrate or recombinant factor VIII</p> Signup and view all the answers

    Which of the following is a characteristic of the inheritance pattern of hemophilia A?

    <p>It is inherited as an X-linked recessive trait</p> Signup and view all the answers

    What is the primary function of the Partial Thromboplastin Time (PTT) test?

    <p>Assesses intrinsic clotting pathway activity</p> Signup and view all the answers

    Which clotting factors affect both Partial Thromboplastin Time (PTT) and Prothrombin Time (PT)?

    <p>Prothrombin (II) and Fibrinogen (I)</p> Signup and view all the answers

    Which symptom is NOT commonly associated with patients having problems in primary hemostasis?

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

    What clinical presentation is typical for patients with primary hemostasis issues?

    <p>Intracranial bleeding with severe thrombocytopenia</p> Signup and view all the answers

    Which laboratory test measures the final step in the clotting cascade?

    <p>Thrombin time (TT)</p> Signup and view all the answers

    Which of the following is a feature of primary hemostasis problems?

    <p>Epistaxis and GI bleeding</p> Signup and view all the answers

    What distinguishes von Willebrand disease (vWD) from classic Hemophilia A?

    <p>Response to Desmopressin (DDAVP)</p> Signup and view all the answers

    Which type of von Willebrand disease (vWD) is the most common?

    <p>Type 1</p> Signup and view all the answers

    What is a characteristic laboratory finding in Bernard Soulier syndrome?

    <p>Normal platelet aggregation in response to vWF</p> Signup and view all the answers

    How do individuals with von Willebrand disease (vWD) typically respond to Desmopressin (DDAVP)?

    <p>Increased vWF and factor VIII levels</p> Signup and view all the answers

    What distinguishes the bleeding manifestations between classic Hemophilia A and von Willebrand disease?

    <p>Type of hemorrhage</p> Signup and view all the answers

    What distinguishes Bernard Soulier syndrome from other platelet disorders?

    <p>Platelet aggregation in response to vWF</p> Signup and view all the answers

    What is the primary defect in Glanzmann's thrombasthenia?

    <p>Genetic GIIB/IIIA deficiency leading to impaired platelet aggregation</p> Signup and view all the answers

    What is the most common inherited cause of hypercoagulability?

    <p>Factor V Leiden mutation</p> Signup and view all the answers

    At what platelet count level can fatal CNS or GI hemorrhage occur?

    <p>Around 5x10^3/µl</p> Signup and view all the answers

    What is the characteristic of thrombocytopenia?

    <p>Prolonged bleeding time with normal PT and PTT</p> Signup and view all the answers

    What is the typical clinical presentation of acute idiopathic (childhood) thrombocytopenic purpura (ITP)?

    <p>Develops acutely within 1-2 weeks with a short duration</p> Signup and view all the answers

    At what platelet count level may bleeding occur, depending on the platelets' functional status?

    <p>Between 20-60x10^3/µl</p> Signup and view all the answers

    Which of the following is the most common cause of chronic Immune Thrombocytopenic Purpura (ITP) in adults?

    <p>Idiopathic (unknown cause)</p> Signup and view all the answers

    What is the characteristic finding on peripheral blood smear in Immune Thrombocytopenic Purpura (ITP)?

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

    Which of the following is the most common initial treatment for severe cases of Immune Thrombocytopenic Purpura (ITP)?

    <p>High-dose intravenous immunoglobulins</p> Signup and view all the answers

    What is the underlying mechanism in Immune Thrombocytopenic Purpura (ITP)?

    <p>Formation of anti-platelet antibodies</p> Signup and view all the answers

    Which of the following is the characteristic bone marrow finding in Immune Thrombocytopenic Purpura (ITP)?

    <p>Increased megakaryocyte numbers</p> Signup and view all the answers

    What is the underlying pathology in microangiopathic thrombocytopenia?

    <p>Formation of platelet microthrombi in small blood vessels</p> Signup and view all the answers

    What is the most common bleeding site in patients with hemophilia A?

    <p>Muscles and joints</p> Signup and view all the answers

    What is the primary defect in Glanzmann's thrombasthenia?

    <p>Deficiency in platelet glycoprotein IIb/IIIa complex</p> Signup and view all the answers

    What is the most appropriate treatment for hemophilia A?

    <p>Factor VIII concentrate or recombinant VIII replacement therapy</p> Signup and view all the answers

    Which type of von Willebrand disease (vWD) is the most common?

    <p>Type 1</p> Signup and view all the answers

    What is the role of von Willebrand factor in hemophilia A?

    <p>It stabilizes and protects factor VIII from degradation</p> Signup and view all the answers

    What is the typical laboratory finding in hemophilia A?

    <p>Prolonged activated partial thromboplastin time (aPTT)</p> Signup and view all the answers

    What is the most common type of von Willebrand disease (vWD)?

    <p>Type 1</p> Signup and view all the answers

    Which of the following is NOT a characteristic of von Willebrand disease (vWD)?

    <p>Hemarthroses and deep muscle hemorrhage</p> Signup and view all the answers

    How do individuals with von Willebrand disease (vWD) typically respond to Desmopressin (DDAVP) treatment?

    <p>DDAVP temporarily increases vWF and factor VIII levels 2-3 times</p> Signup and view all the answers

    What is the characteristic laboratory finding in Bernard Soulier syndrome?

    <p>All of the above</p> Signup and view all the answers

    Which of the following is a distinguishing feature between classic Hemophilia A and von Willebrand disease (vWD)?

    <p>All of the above</p> Signup and view all the answers

    Which type of von Willebrand disease (vWD) has the most severe bleeding?

    <p>Type 3</p> Signup and view all the answers

    What is the purpose of the bleeding time test?

    <p>To assess platelet function by timing how long it takes for bleeding to stop</p> Signup and view all the answers

    Which of the following is a common symptom of patients with primary hemostasis problems?

    <p>Hematuria (blood in urine)</p> Signup and view all the answers

    What is measured by the Prothrombin Time (PT) and International Normalized Ratio (INR) tests?

    <p>Activity of the extrinsic clotting pathway</p> Signup and view all the answers

    Which of the following is a common skin manifestation in patients with primary hemostasis problems?

    <p>Ecchymoses (larger than 1cm bleeding spots)</p> Signup and view all the answers

    Which of the following clotting factors affect both the Partial Thromboplastin Time (PTT) and Prothrombin Time (PT)?

    <p>Factor X, Factor V, Prothrombin (Factor II), and Fibrinogen (Factor I)</p> Signup and view all the answers

    Which laboratory test measures the final step in the clotting cascade, where fibrinogen is converted to fibrin?

    <p>Thrombin Time (TT)</p> Signup and view all the answers

    What is the most common cause of chronic Immune Thrombocytopenic Purpura (ITP) in adults?

    <p>Idiopathic or secondary to conditions like SLE or HIV</p> Signup and view all the answers

    What is the characteristic finding on peripheral blood smear in Immune Thrombocytopenic Purpura (ITP)?

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

    Which of the following is the most common initial treatment for severe cases of Immune Thrombocytopenic Purpura (ITP)?

    <p>High-dose intravenous immunoglobulins</p> Signup and view all the answers

    What is the underlying mechanism in Immune Thrombocytopenic Purpura (ITP)?

    <p>Formation of anti-platelet antibodies, primarily IgG against GPIIb/IIIa</p> Signup and view all the answers

    Which of the following is the characteristic bone marrow finding in Immune Thrombocytopenic Purpura (ITP)?

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

    What is the underlying pathology in microangiopathic thrombocytopenia?

    <p>Platelet microthrombi formation in small blood vessels</p> Signup and view all the answers

    What is a key difference between Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP)?

    <p>TTP commonly presents with neurological symptoms, while HUS is limited to kidney involvement.</p> Signup and view all the answers

    What distinguishes Disseminated Intravascular Coagulation (DIC) from Hemolytic Uremic Syndrome (HUS)?

    <p>HUS is a primary disease, whereas DIC is a complication of conditions with endothelial cell damage.</p> Signup and view all the answers

    What is a typical laboratory finding in patients with Disseminated Intravascular Coagulation (DIC)?

    <p>Elevated D-dimers and other fibrin degradation products</p> Signup and view all the answers

    Which of the following statements regarding Hemolytic Uremic Syndrome (HUS) is accurate?

    <p>HUS can follow viral or bacterial infections and predominantly affects children.</p> Signup and view all the answers

    What distinguishes Disseminated Intravascular Coagulation (DIC) from Thrombotic Thrombocytopenic Purpura (TTP)?

    <p>DIC leads to thrombo-hemorrhagic disorder, while TTP does not involve bleeding.</p> Signup and view all the answers

    Which laboratory finding is characteristic of patients with Disseminated Intravascular Coagulation (DIC)?

    <p>Low levels of clotting factors</p> Signup and view all the answers

    Which coagulation factor is deficient in individuals with hemophilia B?

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

    What is the most common inheritance pattern of von Willebrand disease (vWD)?

    <p>Autosomal dominant</p> Signup and view all the answers

    What is the role of von Willebrand factor (vWF) in the coagulation process?

    <p>It stabilizes factor VIII</p> Signup and view all the answers

    Which laboratory test is used to diagnose von Willebrand disease (vWD)?

    <p>Ristocetin-induced platelet aggregation (RIPA)</p> Signup and view all the answers

    What is the most common initial treatment for severe cases of hemophilia B?

    <p>Recombinant factor IX concentrates</p> Signup and view all the answers

    Which of the following is a characteristic of the inheritance pattern of hemophilia B?

    <p>X-linked recessive</p> Signup and view all the answers

    What is the primary role of factor VIII in the coagulation cascade?

    <p>Activates factor X, which then activates prothrombin</p> Signup and view all the answers

    Which of the following is a characteristic clinical manifestation of hemophilia A?

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

    What is the underlying genetic defect in hemophilia A?

    <p>X-linked recessive mutation in the factor VIII gene</p> Signup and view all the answers

    Which laboratory test is typically prolonged in patients with hemophilia A?

    <p>Partial thromboplastin time (PTT)</p> Signup and view all the answers

    What is the primary treatment for hemophilia A?

    <p>Factor VIII replacement therapy</p> Signup and view all the answers

    Which of the following statements is true regarding the inheritance pattern of hemophilia A?

    <p>It is an X-linked recessive disorder that almost exclusively affects males</p> Signup and view all the answers

    What is the underlying mechanism in Immune Thrombocytopenic Purpura (ITP)?

    <p>Formation of anti-platelet antibodies, IgG against platelet glycoprotein IIb/IIIa</p> Signup and view all the answers

    What is the underlying pathology in microangiopathic thrombocytopenia?

    <p>Formation of platelet microthrombi in small blood vessels that mechanically damage red blood cells</p> Signup and view all the answers

    Which of the following is the most common initial treatment for severe cases of Immune Thrombocytopenic Purpura (ITP)?

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

    What is the characteristic laboratory finding on peripheral blood smear in Immune Thrombocytopenic Purpura (ITP)?

    <p>Increased numbers of large platelets</p> Signup and view all the answers

    What is the underlying cause of chronic Immune Thrombocytopenic Purpura (ITP) in adults?

    <p>Autoimmune disorders like systemic lupus erythematosus (SLE)</p> Signup and view all the answers

    What is the characteristic bone marrow finding in Immune Thrombocytopenic Purpura (ITP)?

    <p>Increased number of megakaryocytes</p> Signup and view all the answers

    What is the most common causative agent in Hemolytic Uremic Syndrome (HUS)?

    <p>Toxin-induced endothelial damage</p> Signup and view all the answers

    Which laboratory test measures the final step in the clotting cascade, where fibrinogen is converted to fibrin?

    <p>Prothrombin Time (PT)</p> Signup and view all the answers

    What distinguishes Hemolytic Uremic Syndrome (HUS) from Thrombotic Thrombocytopenic Purpura (TTP)?

    <p>E.coli O157:H7 as a causative agent</p> Signup and view all the answers

    What distinguishes the bleeding manifestations between Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS)?

    <p>Platelet microaggregate formation</p> Signup and view all the answers

    Which laboratory finding is common in both Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP)?

    <p>Normal Prothrombin Time (PT)</p> Signup and view all the answers

    Which of the following is a key distinguishing feature between von Willebrand disease (vWD) and classic hemophilia A?

    <p>All of the above.</p> Signup and view all the answers

    Which of the following statements about von Willebrand disease (vWD) types is correct?

    <p>Type 3 vWD is the most severe form and patients do not respond to desmopressin (DDAVP) treatment.</p> Signup and view all the answers

    Which of the following statements regarding the treatment of von Willebrand disease (vWD) is correct?

    <p>Desmopressin (DDAVP) can be used to temporarily increase vWF and factor VIII levels 2-3 times in patients with Type 1 and Type 2 vWD.</p> Signup and view all the answers

    Which of the following laboratory findings is characteristic of Bernard-Soulier syndrome?

    <p>All of the above</p> Signup and view all the answers

    Which of the following statements regarding the pathophysiology of Immune Thrombocytopenic Purpura (ITP) is correct?

    <p>In ITP, autoantibodies bind to and destroy platelets, resulting in thrombocytopenia.</p> Signup and view all the answers

    Which of the following statements regarding the laboratory findings in Disseminated Intravascular Coagulation (DIC) is correct?

    <p>Patients with DIC typically exhibit thrombocytopenia and the presence of schistocytes on peripheral blood smear.</p> Signup and view all the answers

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