30 Questions
What is the primary pathogenic mechanism in acquired TTP?
Autoantibody against ADAMTS 13
Which of the following is NOT a characteristic feature of TTP?
Acute renal failure
What is the primary pathogenic mechanism in HUS?
E.coli O157:H7 toxin-induced endothelial damage
Which laboratory test is typically normal in TTP and HUS?
Prothrombin time (PT)
What is the primary treatment for TTP?
Immunosuppressive agents
Which of the following is a key difference between TTP and HUS?
Presence of neurological symptoms
What is the primary method used to diagnose von Willebrand disease?
Using the ristocetin platelet agglutination test
What is the mechanism behind vWF-mediated platelet agglutination during the ristocetin test?
Formation of interplatelet bridges
What characterizes type IIA von Willebrand disease?
Normal multimers but rapid removal from circulation
What is primarily affected in hemophilia A?
Factor VIII activity
In hemophilia A, what level of factor VIII activity defines severe cases?
Activity levels < 1% of normal
What is the X-linked recessive disorder primarily caused by reduced factor VIII activity?
Hemophilia A
Which of the following best describes the pathogenesis of Immune Thrombocytopenic Purpura (ITP)?
Antibodies against platelet membrane glycoproteins IIb/IIIa or Ib/IX complexes
Which of the following statements about splenectomy in ITP is true?
Splenectomy normalizes the platelet count and induces complete remission in more than two-thirds of patients
What is a common finding in the bone marrow of patients with ITP?
Increased numbers of megakaryocytes
Which of the following statements about splenomegaly in ITP is true?
Splenomegaly is not a feature of uncomplicated ITP
Which of the following clinical manifestations is uncommon in ITP?
Intracerebral or subarachnoid hemorrhages
Which of the following statements about the diagnosis of ITP is true?
The diagnosis rests on the clinical features, thrombocytopenia, examination of the marrow, and the exclusion of secondary ITP
What is the main consequence of widespread fibrin deposition within the microcirculation in DIC?
Obstruction leading to ischemia in severely affected or vulnerable organs
Which of the following can trigger DIC according to the text?
Bacterial sepsis
What is the primary pathologic activation related to the depletion of elements required for hemostasis in DIC known as?
Consumptive coagulopathy
What kind of anemia is associated with DIC due to RBC trauma while passing through vessels narrowed by fibrin thrombi?
Microangiopathic hemolytic anemia
Which of the following is NOT a trigger for Disseminated Intravascular Coagulation according to the text?
Normal pregnancy
What causes the secondary release of plasminogen activator in DIC?
Depletion of platelets and clotting factors
What is the primary source of plasma von Willebrand factor (vWF)?
Endothelial cells
What is the primary function of factor VIII in the coagulation pathway?
Activates factor IX
What is the most common inherited bleeding disorder?
von Willebrand disease
What is the primary clinical manifestation of von Willebrand disease?
All of the above
Which subtype of von Willebrand disease is characterized by the selective loss of high-molecular-weight multimers of vWF?
Type II
What is the primary reason why von Willebrand disease is underrecognized?
The diagnosis requires sophisticated tests
Learn about thrombocytopenia, its clinical importance, and the two clinical subtypes of Immune Thrombocytopenic Purpura (ITP). Understand the pathogenesis involving antibodies against platelet membrane glycoproteins. Explore how larger hemorrhages in the CNS are associated with marked thrombocytopenia.
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