Coagulation

Question 1

what are the clotting factors

Answer 1

platelets
soluble clotting
tissue factor
collagen & phospholipids

Question 2

what are anti-clotting factors

Answer 2

Nitric oxide
Protein C&S
Anti-thrombin
fibrinolytics
protacyclin

Question 3

what is protime or PT

Answer 3

sensitively index measures the concentration of clotting factors in the extrinsic pathway and the common pathway, may which depend on Vit K for their activity

Question 4

what is the activated partial thromboplastin time aPTT

Answer 4

is most sensitive to the concentration of clotting factors in the intrinsic pathway and the common pathway

Question 5

What is Von Willebrand disease

Answer 5

most common bleeding disorder
Von Willebrand binds factor VIII and extends life of VIII
binds platelets to injured vessel endothelium
helps to insure that coagulation and healing take place at site of vessel injury

Question 6

what is the clinical presentation of Von Willebrand

Answer 6

mucosal bleeding including epistaxis & after dental extraction
Post Op bleeding
Metromenorrhagia
Hemarthrosis

Question 7

what is a critical step in the clotting cascade

Answer 7

factor X to Xa conversion

Question 8

what does proteins C&S do

Answer 8

inhibits the feedback pathway for factor V

Question 9

factor II is also known as

Answer 9

prothrombin

Question 10

protime most sensitively measures which pathway and which factors

Answer 10

common pathway

which depends on Vitamin K for their activity: II, VII, IV, X

Question 11

aPTT is most sensitive for which pathway?

Answer 11

intrinsic pathway and the common pathway

F XI, IX, VIII, II prothrombin

Question 12

how is heparin measured

Answer 12

anti Xa

Question 13

what is the tip off lab test for Von willibrand dz

Answer 13

increased aPTT

Question 14

what is the most common type of Von Willebrand dz

Answer 14

Type 1

autosomal co-dominant

Question 15

what is the best way to treat mild Von Willebrand dz

Answer 15

DDAVP which can be given IV or Nasal spray

Question 16

More severe bleeding require what to tx VWB dz

Answer 16

factor replacement with cryoprecipitate

Question 17

what is different about VWB type 2b

Answer 17

has excessively big multimers and be associated with thrombocytopenia and SHOULD NOT BE TREATED WITH DDAVP b/c this can worsen thrombocytopenia

Question 18

Hemophilia A

Answer 18

x-linked, therefore only seen in males
Deficiency of FVIII
manifests as easy bruising or bleeding particularly hemarthoses

Question 19

how is hemophilia A treated

Answer 19

recombinant factor VIII replacement IV

Question 20

hemophilia B

Answer 20

x linked recessive therefore severe Sx only in males

factor IX deficiency

Question 21

how is hemophilia B Tx

Answer 21

recombinant IX

Question 22

what is Factor XI

Answer 22

autosomal co-dominant
+Fam HX
rare in gen public but as much a 6% of ashkenazi Jews
Only few are Symptomatic and most present in adulthood

Question 23

how is factor XI tx

Answer 23

fresh frozen plasma