Pathology · Platelet and Coagulation Disorders

A 35-year-old man with severe hemophilia A (factor VIII <1%) presents with a life-threatening retroperitoneal hemorrhage. He has a history of factor VIII inhibitors. Laboratory shows a Bethesda titer of 35 BU. Which bypassing agent is most appropriate?

  • A Recombinant activated factor VII (rFVIIa)
  • B Fresh frozen plasma transfusion
  • C Recombinant factor VIII concentrate at high dose
  • D Cryoprecipitate infusion
Correct answer: A. Recombinant activated factor VII (rFVIIa)

Explanation

High-titer inhibitors (>5 BU) render factor VIII replacement ineffective because the antibody neutralizes infused factor. Bypassing agents such as recombinant activated factor VII (rFVIIa) or activated prothrombin complex concentrate (aPCC) generate thrombin independently of the intrinsic tenase complex. rFVIIa directly activates factor X on tissue factor-bearing cells at the site of injury. FFP and cryoprecipitate provide insufficient factor VIII and would be neutralized.

Reference: Williams Hematology, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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