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 (C)</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 (B)</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 (D)</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 (A)</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.) (B)</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 (B)</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 (D)</p> Signup and view all the answers

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

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

What is the typical laboratory finding in hemophilia A?

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

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

<p>Hepatomegaly (D)</p> Signup and view all the answers

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

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

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

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

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

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

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

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

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

<p>Type 1 (C)</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 (D)</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 (B)</p> Signup and view all the answers

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

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

What distinguishes Bernard Soulier syndrome from other platelet disorders?

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

What is the most common inherited cause of hypercoagulability?

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

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

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

What is the characteristic of thrombocytopenia?

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

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

<p>Large platelets (C)</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 (D)</p> Signup and view all the answers

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

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

What is the underlying pathology in microangiopathic thrombocytopenia?

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

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

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

What is the primary defect in Glanzmann's thrombasthenia?

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

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

<p>Type 1 (D)</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 (C)</p> Signup and view all the answers

What is the typical laboratory finding in hemophilia A?

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

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

<p>Type 1 (A)</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 (D)</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 (D)</p> Signup and view all the answers

What is the characteristic laboratory finding in Bernard Soulier syndrome?

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

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

<p>Type 3 (A)</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 (C)</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) (D)</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 (C)</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) (B)</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) (A)</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) (A)</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 (D)</p> Signup and view all the answers

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

<p>Large platelets (C)</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 (D)</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 (B)</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 (B)</p> Signup and view all the answers

What is the underlying pathology in microangiopathic thrombocytopenia?

<p>Platelet microthrombi formation in small blood vessels (D)</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. (B)</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. (A)</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 (B)</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. (B)</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. (A)</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 (D)</p> Signup and view all the answers

Which coagulation factor is deficient in individuals with hemophilia B?

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

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

<p>Autosomal dominant (B)</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 (A)</p> Signup and view all the answers

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

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

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

<p>Hemarthrosis and muscle hematomas (C)</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 (A)</p> Signup and view all the answers

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

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

What is the primary treatment for hemophilia A?

<p>Factor VIII replacement therapy (D)</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 (C)</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 (A)</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 (D)</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 (B)</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 (A)</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) (A)</p> Signup and view all the answers

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

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

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

<p>Toxin-induced endothelial damage (C)</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) (B)</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 (A)</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 (B)</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) (D)</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. (D)</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. (B)</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. (D)</p> Signup and view all the answers

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

<p>All of the above (D)</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. (B)</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. (B)</p> Signup and view all the answers

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