Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
17 Cards in this Set
- Front
- Back
|
What are the sources of vonWillebrand factor?
|
85% in endothelium and 15% in plt/megakaryocytes
|
|
|
What kind of inherited disease is it?
|
autosomal dominant
|
|
|
What is the structure of vWF?
|
large repeating multimers of glycoprotein
|
|
|
What is circulating vWF associated w/?
|
non-covalently associated with VIII:C (f8)
|
|
|
What is the vWF/VIII:C complex?
|
it consists of 95% vWF and protects VIII:C from inactivation and removal
|
|
|
What are the 2 variants of vW disease?
|
1.) Acquired and
2.) Pseudo-vW disease |
|
|
What is acquired vW disease?
|
- it is usually severe w/ less than 20% factor activity
- it is associated with lupus and myeloproliferative diseases - as a result of a circulating antibody against vWF |
|
|
What is Pseudo-vW disease?
|
- it is rare
- it is actually a plt abnormality causing spontaneous aggregation which lowers vWF in the process |
|
|
What is type I vW disease?
|
- reduced amount of all multimers
- usually plt vWF is normal |
|
|
What is type II vW disease?
|
- abnormal multimers and partial reduction
|
|
|
What is type IIa?
|
HMW multimers reduced
|
|
|
What is type IIb?
|
abnormal HMW w/ increased affinity for plts.
|
|
|
What is type III?
|
- severe reduction in all multimers
- plt vWF reduced as well |
|
|
What are the treatments for vW disease?
|
1.) 1-Deamino-8-D-Argenine Vasopressin (DDAVP)
2.) Cryoprecipitate 3.) Factor VIII concentrate |
|
|
What is DDAVP?
|
- squeeze endothelial and get vWF out
- temporary increase in vWF - for type I and IIA |
|
|
What is cryoprecipitate?
|
- contains 40-70% of original FFP
- the leftover on the walls after de-thaw |
|
|
What is Factor VIII concentrate?
|
- not all F8 concentrates contain vWF, be sure to consult product insert
|
