Factor XIII deficiency in a test question

[ChessMaster3000]

I'm wondering how a patient might present with factor 13 deficiency. I read somewhere else that the "classic" question would be continued bleeding despite normal values for all bleeding parameters (BT, PT, PTT, platelet count). can someone verify this? Has anyone seen factor 13 come up on any questions and if so, how did they present?

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[notbobtrustme]

well, according to wikipedia, it's just another crosslinker of fibrin. I'm not too sure what would happen, but it does sound like normal PT/PTT/bleeding time would be expected since there is no inherent defect in platelet aggregration/adhesion nor the coagulation pathway. So yea, F-13 deficiency might present with continued bleeding despite normal lab values.

Stressing out about it seems super low yield though.

 

[Myxedema]

What you've written is correct: Factor XIII deficiency is a rare bleeding disorder with normal coagulation labs. Master the Boards books (both the Step 2 and Step 3 version) explain them wellh, please refer to those books if you want more information. As a summary for these rare coagulation disorders:
- Factor XI deficiency: Clinical: Bleeding after trauma/surgery. Labs: N PT, elevated aPTT.
- Factor XII deficiency: Clinical: No bleeding. Labs: N PT, elevated aPTT.
- Factor XIII deficiency: Clinical: Bleeding after trauma/surgery. Labs: N PT, N aPTT