Secondary. Hemostasis Disorders Flashcards
What is going on with hemophilia A, what is the problem, what causes it and what is the main effect?
Factor 8 deficiency either by an x linked recessive mutation or de novo mutation.
Increased deep bleeding.
3 lab values to remember about hemophilia A and what is the treatment?
Increased PTT and normal PT
Low factor 8
Normal platelets and bleeding time
Give factor 8
What is hemophilia B and what is the other name for it?
Christmas disease.
Same as hemophilia a but this is factor 9 problem and not 8.
What is coagulation factor inhibitor and how do we distinguish it from hemophilia A?
Acquired antibody against a coagulation factor and 8 is most common. Everything will be the same as hemophilia 8 so we have to do the mixing study.
In hemophilia 8, when we mix normal plasma with patient’s plasma the PTT will recorrect because it has 8 now because the problem was just deficiency.
In coagulation factor inhibition, when we mix the patients blood with normal plasma, the antibodies will destroy those factor 8’s as well and the PTT will remain high.
What is the most common inherited coagulation disorder, what is the most common type, what is the effect?
VWF deficiency.
Autosomal dominant
Impairs platelet adhesion causing mucosal and skin bleeding
What are the three lab values for vWF disease?
Bleeding time is up
PTT is up because vWF stabilizes factor 8
Abnormal ristocetin test because when you give ristocetin it causes platelet aggregation by promoting vWF to bind platelets via 1b, but if you don’t have vWF this ain’t happening.
How do we treat vWF disease and why?
Give desmopressin because it promotes vWF release from WP bodies of endothelial cells
What is the role of vitamin K deficiency in clotting problems? what are three main ways we get vitamin k deficiency?
Vitamin K is activated by epoxide reductase in the liver and then helps activate 2,7,9,and 10 and proteins c and s.
Newborns, long term antibiotic therapy and malabsorption (fat soluble vitamins, k being one).
What are 2 “other” causes of abnormal secondary hemostasis?
Liver failure because we aren’t able to make the clotting factors and epoxide reductase
Large volume transfusions that dilute out the clotting factors.
