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ICC2 > von willibrand's disease > Flashcards

von willibrand's disease Flashcards

1
Q

what are the two types of factors in VWD?

A

tubular vWF

filamentous vWF

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2
Q

where is the tubular vWF found ?

A

sub endothelium

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3
Q

what is the function of the filamentous vWF?

A

trapping mesh

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4
Q

what is the pattern of inheritance inn VWD ?

A

can be autosomal recessive or dominant and can affect both males and females

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5
Q

what are the types of VWD ?

A

type 1
type 2
type 3

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6
Q

what is the defect in type 1?

A

mild quantitive

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7
Q

what is the method of inheritance in type 1 ?

A

autosomal dominant with incomplete penetrance

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8
Q

what are the lab abnormalities in type 1 ?

A

increased APTT
decreased VWF-ag
decreased VWF- ac
equal Ag and Ac

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9
Q

what is the treatment for type 1 VWD?

A
  • DDAVP

- Tranexamic acid

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10
Q

what is the defect inn type 2 VWD?

A

qualitative

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11
Q

what is the inheritance pattern in VWD type 2 ?

A

autosomal dominant/recessive

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12
Q

what are the lab abnormalities in type 2?

A

increased APTT
decreased VWF-ag
extremely low VWF-Ac
Ag>Ac

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13
Q

what is the treatment for type 2 ?

A
  • DDAVP (desmopressin)
  • tranexamic acid
  • concentrate
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14
Q

what is the defect inn type 3 VWD?

A

severe quantitative

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15
Q

what is the method of inheritance of Type 3 VWD ?

A

autosomal recessive

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16
Q

what are the lab abnormalities in type 3 VWD?

A

increased APTT
extremely low VWF-Ag
extremely low VWF-AC
Ag=Ac

17
Q

what is the treatment for type 3 VWD ?

A

concentrate

18
Q

which of the three types is the most common ?

A

type 1

19
Q

which of the three types is the most serious and the rarest ?

A

type 3

20
Q

what investigations are required to make a diagnosis of VWD?

A

initial hemostasis tests

  • CBC and platelete count
  • PT and PTT
  • fibrinogen or TT
21
Q

when do we give packed red blood cell ?

A

in cases of severe bleeding or in extremely low haemoglobin

22
Q

what are the three main treatment options of VWD ?

A

desmopressin (DVAPP)
tranexamic acid
fibrin sealant

23
Q

if the VWD is non responsive to desmopressin what is the next best step in management ?

A

give concentrates along with prophylaxis treatment for bleeding

24
Q

what is DIC ?

A

disseminated intravascular coagulation

theres a problem with both clotting and bleeding

25
Q

what are the common causes of DIC ?

A 
sepsis 
trauma 
malignancy 
obstetric complications
vascular malformation 
reaction to toxins 
immunological disorders
26
Q

what are the lab abnormalities in DIC ?

A 
increased APTT 
increased PT
increased TT 
increased FDP 
decreased fibrinogen 
decreased platelets
27
Q

what are the clinical manifestations of DIC?

A

petechia
purpura
arterial line oozing
venipuncture site bleeding

28
Q

what are the significant lab abnormalities/findings ?

A

d-dimer is high
fibrinogen is low
platelet count is low

29
Q

what is the treatment for DIC ?

A

treat the underlying disorder
platelet transfusion
fresh frozen plasma and cryoprecipitate
coagulation inhibitor concentrate

30
Q

what are the causes of bleeding in liver disorders ?

A

portal hypertension

peptic ulceration

31
Q

how do we make a lab diagnosis of bleeding that has been caused by liver disorders ?

A 
prolonged PTT 
prolonged PTTK 
thrombocytopenia 
normal fibrinogen level 
decreased hepatic stores of vitamin K
32
Q

what is the treatment for prolonged PT/APTT?

A

fresh frozen plasma

prothrombin complex concentrate

33
Q

what is the treatment for low fibrinogen ?

A

cryoprecipitate /fibrinogen concentrate

34
Q

which lab results are an indicator of liver function ?

A

INR/PT

35
Q

what are the vitamin K dependent factors synthesized in the liver ?

A 
factor 2 
7 
9 
10
protein C and Protein S
36
Q

what are the causes of vitamin K deficiency ?

A

obstructive jaundice
chronic diarrhea
liver disease
heamorrhagic states in infants

ICC2 (42 decks)

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