Thrombocytopenia and Immune Thrombocytopenic Purpura (ITP)

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Questions and Answers

What is the primary pathogenic mechanism in acquired TTP?

  • Autoantibody against ADAMTS 13 (correct)
  • Deficiency of metalloprotease ADAMTS 13
  • E.coli O157:H7 toxin-induced endothelial damage
  • Activation of the coagulation cascade

Which of the following is NOT a characteristic feature of TTP?

  • Thrombocytopenia
  • Acute renal failure (correct)
  • Fever
  • Transient neurologic deficits

What is the primary pathogenic mechanism in HUS?

  • Deficiency of metalloprotease ADAMTS 13
  • E.coli O157:H7 toxin-induced endothelial damage (correct)
  • Autoantibody against ADAMTS 13
  • Activation of the coagulation cascade

Which laboratory test is typically normal in TTP and HUS?

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

What is the primary treatment for TTP?

<p>Immunosuppressive agents (B)</p> Signup and view all the answers

Which of the following is a key difference between TTP and HUS?

<p>Presence of neurological symptoms (C)</p> Signup and view all the answers

What is the primary method used to diagnose von Willebrand disease?

<p>Using the ristocetin platelet agglutination test (D)</p> Signup and view all the answers

What is the mechanism behind vWF-mediated platelet agglutination during the ristocetin test?

<p>Formation of interplatelet bridges (D)</p> Signup and view all the answers

What characterizes type IIA von Willebrand disease?

<p>Normal multimers but rapid removal from circulation (D)</p> Signup and view all the answers

What is primarily affected in hemophilia A?

<p>Factor VIII activity (D)</p> Signup and view all the answers

In hemophilia A, what level of factor VIII activity defines severe cases?

<p>Activity levels &lt; 1% of normal (A)</p> Signup and view all the answers

What is the X-linked recessive disorder primarily caused by reduced factor VIII activity?

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

Which of the following best describes the pathogenesis of Immune Thrombocytopenic Purpura (ITP)?

<p>Antibodies against platelet membrane glycoproteins IIb/IIIa or Ib/IX complexes (C)</p> Signup and view all the answers

Which of the following statements about splenectomy in ITP is true?

<p>Splenectomy normalizes the platelet count and induces complete remission in more than two-thirds of patients (B)</p> Signup and view all the answers

What is a common finding in the bone marrow of patients with ITP?

<p>Increased numbers of megakaryocytes (B)</p> Signup and view all the answers

Which of the following statements about splenomegaly in ITP is true?

<p>Splenomegaly is not a feature of uncomplicated ITP (A)</p> Signup and view all the answers

Which of the following clinical manifestations is uncommon in ITP?

<p>Intracerebral or subarachnoid hemorrhages (C)</p> Signup and view all the answers

Which of the following statements about the diagnosis of ITP is true?

<p>The diagnosis rests on the clinical features, thrombocytopenia, examination of the marrow, and the exclusion of secondary ITP (A)</p> Signup and view all the answers

What is the main consequence of widespread fibrin deposition within the microcirculation in DIC?

<p>Obstruction leading to ischemia in severely affected or vulnerable organs (D)</p> Signup and view all the answers

Which of the following can trigger DIC according to the text?

<p>Bacterial sepsis (A)</p> Signup and view all the answers

What is the primary pathologic activation related to the depletion of elements required for hemostasis in DIC known as?

<p>Consumptive coagulopathy (A)</p> Signup and view all the answers

What kind of anemia is associated with DIC due to RBC trauma while passing through vessels narrowed by fibrin thrombi?

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

Which of the following is NOT a trigger for Disseminated Intravascular Coagulation according to the text?

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

What causes the secondary release of plasminogen activator in DIC?

<p>Depletion of platelets and clotting factors (B)</p> Signup and view all the answers

What is the primary source of plasma von Willebrand factor (vWF)?

<p>Endothelial cells (A)</p> Signup and view all the answers

What is the primary function of factor VIII in the coagulation pathway?

<p>Activates factor IX (C)</p> Signup and view all the answers

What is the most common inherited bleeding disorder?

<p>von Willebrand disease (A)</p> Signup and view all the answers

What is the primary clinical manifestation of von Willebrand disease?

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

Which subtype of von Willebrand disease is characterized by the selective loss of high-molecular-weight multimers of vWF?

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

What is the primary reason why von Willebrand disease is underrecognized?

<p>The diagnosis requires sophisticated tests (B)</p> Signup and view all the answers

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