hematologic - coagulation disorders Flashcards
hemophilia A concerns which factor
factor VIII (8)
hemophilia B concerns which factor
factor IX (9)
for surgery, individual clotting factor levels of ____ provide adequate hemostasis
20-25%
what products are available to treat single deficiencies?
factor concentrates, recombinant factors, FFP, gene therapy
how much FFP is needed to obtain a 20-30% increase in the level of any clotting factor?
15-20 mL/kg
hereditary deficiencies
hemophilia A, hemophilia B, von willebrand disease
acquired deficiencies
vitamin k deficiency (antibiotics, intestinal malabsorption, impaired nutrition), liver disease (parenchymal disease), disseminated intravascular coagulation (DIC), autoantibodies
hemophilia A is deficient in factor VIII that affects males or females?
males
severe hemophilia A
<1% factor VIII activity, needs factor VIII concentrate infusions at home
diagnosis of hemophilia A
prolonged aPTT, specific factor testing, and gene testing
anesthesia and hemophilia A
hematology consult, bring factor VIII level >50% prior to surgery, FFP, cryo, TXA
mild - DDAVP 30-90mins prior to surgery
moderate to severe- factor VIII concentrate
half life of Factor VIII
12 hours, but 6 hours in kids so may need redosing for days to weeks after surgery
hemophilia B affects males or females?
males
factor IX levels below ____ are associated with severe bleeding
<1%
mild hemophilia B
levels between 5-40%, isn’t detected until surgery or dental procedure
diagnosis of hemophilia B
prolonged aPTT, specific factor testing, and gene testing
anesthesia and hemophilia B
hematology consult
replacement therapy with recombinant factor IX, purified factor IX, prothrombin complex concentrate
TXA
prothrombin complex concentrate contains which factors
II, VII, IX, X
factor IX half life
18-24 hours
what is the most common congenital bleeding disorder in the world?
von willebrand disease
von willebrand disease is a family of disorders caused by
quantitative and/or qualitative defects