Pathology · Platelet and Coagulation Disorders

A 28-year-old woman presents with her first episode of deep vein thrombosis. She has no obvious risk factors. Laboratory testing shows resistance to activated protein C (APC resistance). Genetic testing confirms a mutation in the factor V gene. What is the molecular mechanism by which factor V Leiden causes thrombophilia?

  • A Factor V cannot be activated by thrombin
  • B Factor V Leiden has increased binding to tissue factor
  • C Factor V Leiden cannot be cleaved and inactivated by activated protein C
  • D Factor V Leiden cannot bind to factor VIII
Correct answer: C. Factor V Leiden cannot be cleaved and inactivated by activated protein C

Explanation

Factor V Leiden has an Arg506Gln mutation at the primary cleavage site for activated protein B. This renders factor V resistant to proteolytic inactivation by APC, so it persists as a procoagulant cofactor, generating excess thrombin. It is the most common inherited thrombophilia in populations of European descent.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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