von willibrand's disease Flashcards
what are the two types of factors in VWD?
tubular vWF
filamentous vWF
where is the tubular vWF found ?
sub endothelium
what is the function of the filamentous vWF?
trapping mesh
what is the pattern of inheritance inn VWD ?
can be autosomal recessive or dominant and can affect both males and females
what are the types of VWD ?
type 1
type 2
type 3
what is the defect in type 1?
mild quantitive
what is the method of inheritance in type 1 ?
autosomal dominant with incomplete penetrance
what are the lab abnormalities in type 1 ?
increased APTT
decreased VWF-ag
decreased VWF- ac
equal Ag and Ac
what is the treatment for type 1 VWD?
- DDAVP
- Tranexamic acid
what is the defect inn type 2 VWD?
qualitative
what is the inheritance pattern in VWD type 2 ?
autosomal dominant/recessive
what are the lab abnormalities in type 2?
increased APTT
decreased VWF-ag
extremely low VWF-Ac
Ag>Ac
what is the treatment for type 2 ?
- DDAVP (desmopressin)
- tranexamic acid
- concentrate
what is the defect inn type 3 VWD?
severe quantitative
what is the method of inheritance of Type 3 VWD ?
autosomal recessive
what are the lab abnormalities in type 3 VWD?
increased APTT
extremely low VWF-Ag
extremely low VWF-AC
Ag=Ac
what is the treatment for type 3 VWD ?
concentrate
which of the three types is the most common ?
type 1
which of the three types is the most serious and the rarest ?
type 3
what investigations are required to make a diagnosis of VWD?
initial hemostasis tests
- CBC and platelete count
- PT and PTT
- fibrinogen or TT
when do we give packed red blood cell ?
in cases of severe bleeding or in extremely low haemoglobin
what are the three main treatment options of VWD ?
desmopressin (DVAPP)
tranexamic acid
fibrin sealant
if the VWD is non responsive to desmopressin what is the next best step in management ?
give concentrates along with prophylaxis treatment for bleeding
what is DIC ?
disseminated intravascular coagulation
theres a problem with both clotting and bleeding
what are the common causes of DIC ?
sepsis trauma malignancy obstetric complications vascular malformation reaction to toxins immunological disorders
what are the lab abnormalities in DIC ?
increased APTT increased PT increased TT increased FDP decreased fibrinogen decreased platelets
what are the clinical manifestations of DIC?
petechia
purpura
arterial line oozing
venipuncture site bleeding
what are the significant lab abnormalities/findings ?
d-dimer is high
fibrinogen is low
platelet count is low
what is the treatment for DIC ?
treat the underlying disorder
platelet transfusion
fresh frozen plasma and cryoprecipitate
coagulation inhibitor concentrate
what are the causes of bleeding in liver disorders ?
portal hypertension
peptic ulceration
how do we make a lab diagnosis of bleeding that has been caused by liver disorders ?
prolonged PTT prolonged PTTK thrombocytopenia normal fibrinogen level decreased hepatic stores of vitamin K
what is the treatment for prolonged PT/APTT?
fresh frozen plasma
prothrombin complex concentrate
what is the treatment for low fibrinogen ?
cryoprecipitate /fibrinogen concentrate
which lab results are an indicator of liver function ?
INR/PT
what are the vitamin K dependent factors synthesized in the liver ?
factor 2 7 9 10 protein C and Protein S
what are the causes of vitamin K deficiency ?
obstructive jaundice
chronic diarrhea
liver disease
heamorrhagic states in infants
