bleeding Flashcards
what is factor I
fibrinogen
what is factor II
prothrombin
what clotting factor protein that is never made In liver
vWF
where is factor V made
liver and megakaryocytes
where is factor 8 made
liver and endothelial cells
where is factor 13 made
liver and macrophages
where is VWF made
endothelial cells and megacaryocytes
all clotting factors are low in infants except these 3
fibrinogen, factor 8, vWF
what factor has the shortest half life
7
what factor hast he longest half life
13
components fo teh contact activation system
HMW kiniogen, prekallikrein, factor 12 and factor 11
what is in platelet alpha granules
proteins- vWF, factor V, fibrinogen
what is the vWF receptor on platelets and disease if you’re missing it
gp1b-9-5 this is the disease is Bernard soulier
what is the fibrinogen receptor on platelets and disease if this is missing
gp1b/2a- disease is glanzmans
thrombin has a direct effect on 6 coag factors. name them
activates 8 to 8a, V to Va, 11 to 11a, fibrinogen to fibrin and then 13 to 13a and TAFI to TAFI a (last two are antifibrionolytic)
what factor cross links fibrin
13
what does thrombin do to platelet
activates them
what does thrombomodulin + thrombin do
activates protein C
what does protein C do
inhibits Va and 8a
what does antithrombin do
inhibits thrombin and 10ah
what does protein S do
protein c needs protein S to inhibit 5a and8a
what is PAI1
plasminogen activator inhibitor type 1- inhibits TPA
what does TPA do
plasminogen into plasmin
what is thrombin time testing
fibrinogen to fibrin via thrombin
three factors that are acute phase reactaqnts
fibrinogen, 8, vWF
how does lupus anticoagulant (rarely) cause bleeding
can bind to prothrombin and cause deficiency
what is klippel-trenauny syndrome
large vascular malformation affecting one quadrant of the body
how can peg asparaginase case bleeding
decreased antithrombin, protein S, protein C, and fibrinogen
describe vWD 2a
multimerizastion defect- you dont have the large multimers
describe vWD2b
platelets and vWF stick way TOO well. missing large multimers and if you die ddavp you will drop platelet count
what is vWD 2M
loss of function (opposite of 2b) plt can’t bind VZWD.
what is type 2N vWD
vWD can’t bind factor 8. looks almost like hemophilia
define type 3 vWD
factor and activity are below 10%
what is the only autosomal recessive vWD
type 3
what type of vWD aggregat4es with low dose ristocetin
2B
definition of severe, moderate, and mild for hemophilia
severe is <1, moderate is 1-5, mild is 5-40%
factor 8: 1U/kg increases factor by
TWO percent
factor 9: 1U/kg increases factor by
ONE percent
define high titer hemophilia
more than 5 BU
time points for early, classical, and late for vitamin K def
first 24 hours, 2-7 days, then older than 1 month
factor deficiency to associate with pregnancy loss and spleen rupture
fibrinogen deficiency
factor deficiency common in ashkenazi jews but most patients dont bleed
11
what factor def is most associated with intracranial bleed
13
how does factor XIII present
umbilical stump bleeding
