VWD Flashcards
what is the function of VWF ?
binds and stabilizes factor 8
mediated platelet adhesion
what are the types of VWD ?
type 1 - mild quantitative defect
type 2 - qualitative defect
type 3 - severe quantitative defect
what effect does VWD have on PTT ?
factor 8 is in the intrinsic pathway so it affects the PTT by prolonging it
( isolated increase of PTT )
what is the ratio of Ag to Ac in VWD ?
type 1 - Ag=Ac
type 2 - Ag>Ac
type 3 - Ag=Ac
what is the treatment of each type of VWD ?
Type 1 - DDAVP, tranexamic acid
Type 2- DDAVP, Tranexamic acid concentrate
Type 3- concentrate
which type of VWD is the most common ?
type 1 is the most common
what is the most severe form of the disease ?
type 3
what is the clinical presentation of VWD ?
1- excessive mucocutaneous bleeding
2- which includes spontaneous bruising
recurrent epistaxis
3-bleeding from the gums after brushing teeth
4- menorrhagia in females
what are the tests for hemostasis ?
CBC and platelet count
PT and PTT
Fibrinogen
when can thrombocytopenia be seen in association with VWD ?
in type 2B VWD
what is the treatment for VWD ?
DDVAP
VWF concentrates or recombinant VWF
what is DIC ?
inappropriate stimulation of coagulation leading to thrombotic occlusion of blood vessels
( type of MAHA)
what are the triggers for DIC ?
major trauma or burns
severe sepsis or infection
solid tumors or haematological malignancies
( release of tissue factor into the circulation)
which haematological malignancy is highly associated with DIC ?
APL
when do we perform mixing study ?
after we find out that there is prolonged PTT
what are the lab findings associated with DIC ?
increase in aPTT
increase in PT
increase in TT
decrease in fibrinogen
decrease in platelets
what are the clinical manifestations of DIC ?
petechiae
purpura
arterial line oozing
venipuncture site oozing
what is seen on peripheral blood smear in DIC ?
scchistocytes
when do we generally see schistocytes ?
MAHA
microangiopathic hemolytic anemias
what are the two phases associated with DIC ?
early hypercoagulable state
clinical manifestation phasee
what is the treatment of DIC ?
treatment of the underlying disorder
platelet transfusion
FFP
what are the factors made by the liver that inhibit coagulation ?
antithrombin 3
protein C and protein S
what is the management of hemostatic defects in liver disease ?
1- treatment of prolonged PT and PTT by given fresh frozen plasma
prothrombin complex concentrate
2- treatment of low fibrinogen
what are thee vitamin K dependant factors ?
factor 2,7,9,10
protein C and protein S
what are the causes of vitamin K deficiency ?
obstructive jaundice
chronic diarrhea
liver disease
hemorrhagic states in infants
