anticoagulating disorders

Question 1

hemophilia

Answer 1

most common hereditary disease
hemophilia A and B
both sex-linked recessive
associated w/ HIV and hep c
risk of intracranial hemorrhage worsens prognosis

Question 2

hemophilia A

Answer 2

deficient in fact 8
easy bruising
massive bleeding with trauma and surgery
hemarthroses (bleeding into joints)
no petechiae
normal bleeding time, platelet count, PT
increased PTT
factor 8 assay
factor 8 replacement

Question 3

hemophilia B

Answer 3

factor 9 deficiency
less common
x-linked recessive
no petechiae
normal bleeding time, platelet count, PT
increased PTT
factor 9 assay
factor 9 replacement

Question 4

von willebrand disease

Answer 4

most common bleeding disorder
autosomal dominant
type 1 most common
easy bruising
mucosal surface bleeding (GI)
menorrhagia
NSAIDS and ASA worsen symps
increased bleeding time
decreased factor 8 acitivity
give high-purity factor 8 containing von willebrand factor
PLT transfusions
DDAVP (desmopressin)

Question 5

deficiencies of vitamin K dependent factors

Answer 5

affects clotting factors 2, 7, 9, and 10
autosomally inherited
prolonged bleeding of umbilical cord stump
factor 7 deficiency= increased PT/INR and normal aPPT
factor 2 or 10 deficiency= increased PT/INR and aPPT
transfusion

Question 6

causes of vit. K deficiency

Answer 6

eradication of gut flora (antibiotics)
nutritional deficiency (critical illness or TPN)
malabsorption of fat soluble vitamins or small bowel (celiac sprue or IBD)

Question 7

diagnosis and treatment of vitamin K deficiency

Answer 7

PT and INR
oral vit. K
parenteral vit. K
transfusion

Question 8

liver failure and bleeding

Answer 8

impairs activation of vitamin K dependent clotting factors (2, 7, 9, 10)
portal hypertension causes pooling of platelets in spleen leading to thrombocytopenia-> can lead to GI bleed

Question 9

bleeding w/ renal insufficiency

Answer 9

purpura, epistaxis, GI bleeding
normal PLT
hemodialysis

Question 10

DIC

Answer 10

disseminated intravasuclar coagulation
caused via malignancy, infection, pregnancy
thrombin, fibrin, and platelets become activated
rapid thrombosis consumes factors faster than they can be replaced
hemostasis->coagulation->hemorrhage

Question 11

DIC labs

Answer 11

increased PT/INR
increased aPPT
thrombocytopenia
increased fibrin degradation products
schistocytes

Question 12

treatment of DIC

Answer 12

platelet and FFP transfusion
cryroprecipitate
PRBCs
antifibrinolytic agents are contraindicated

Question 13

hypercoaguable states

Answer 13

thrombophilias
increased risk of thrombosis
genetic
deficiency of anti-thrombic factor (antithrombin 3)
increase in prothrombic factor (hyperhomocysteinemia)->atherosclerosis and VTE
treated via TPA (dissolves blood clots)
plavix/asprin
refer to hematologist
coumadin/xarelto/lovenox (pregnancy)

Question 14

general treatment of bleeding

Answer 14

FFP
cryoprecipitate
vit. K

Question 15

vit. K and FFP

Answer 15

reverse coumadin

Question 16

protamine sulfate

Answer 16

reverses heparin