Acquired/Inheritied Coagulopathy Flashcards
What factor deficiency is associated with amyloidosis?
Acquired Factor X-it’s deficient because it pulled out of the circulation by amyloid fibrils.
What rare factor deficiency can be seen with lupus inhibitor?
Prothrombin or factor II deficiency.
What are the clinical features of factor VII def? How do you treat?
Spontaneous bleeding and bleeding after surgery/trauma (intracranial bleed). Autosomal recessive. PT alone will be elevated. PTT, TT, and Reptilase time will be normal. Tx-novo seven and fibrinolytics. Can also consider PCC and FFP.
What complications besides bleeding are those with Factor VII def at risk of?
DVT/PE
What plasma dyscrasias are assoc w/factor X def?
Multiple Myeloma and Amyloid.
What are the clinical features seen with factor X def?
Severe bleeding-mucocutaneous/soft tissue bleed. Very high risk of intracranial bleed. Prolonged PT/INR and PTT. Normal TT and Reptiplase time.
What is the treatment of factor X def?
Factor X plasma, PCC, FFP
Do factor X levels in factor X def closely correlate with bleeding severity?
Yes!
Factor V def can be associated with what other factor def and what gene mutations cause this?
It can be associated with factor VIII deficiency in the presence of a LMAN1 or MCFD2 mutation
What are acquired causes of factor V def? What is the treatment?
Topical bovine thrombin, Quebec Platelet Syndrome, autoantibodies can also form. FFP and platelet transfusion.
What are the clinical features with Factor XIII def? Does factor level correspond to bleeding symptoms?
Patients will have severe bleeding symptoms (e.g. umbillical stump, ICH). Also have poor wound healing and pregnancy loss (miscarriage). Factor level corresponds to bleeding severity.
How do we treat Factor XIII def?
A subunit missing-recombinant factor XIII
B subunit missing-plasma derived factor XIII or FFP or cryoprecipitate.
How do you diagnose factor XIII def?
Clot solubility test and factor XIII level. Will need genotype analysis to see which subunit is defected.
Factor II def can develop due to an autoantibody from what? How will this specifically present?
This can develop related to antibody from APS. Both the PT and PTT will be prolonged, however the mixing study will correct the PT, but not the PTT.
What are the clinical features of factor II deficiency and how do you diagnose these patients?
Severe mucocutaneous, intramuscular, or resulting from trauma/surgery. Can also develop hemarthrosis. You diagnose by: TT, factor activity assay, prothrombin antigen level, genotyping.
