von Willebrand Disease Flashcards
What is von Willebrand disease, how common is it,
and does it affect males or females more often?
von Willebrand disease is an inherited platelet function disorder
caused by a deficiency in von Willebrand factor that affects
males and females equally and occurs in about 1% of the
population
Longnecker DE, Newman MF, Brown DL, Zapol WM.
Anesthesiology. 2nd ed. New York: McGraw-Hill; 2012: 209.
What is the function of von Willebrand factor?
It serves as a carrier for factor VIII and acts to protect it from
proteolytic cleavage. It also binds platelet receptors to exposed
collagen initially by a Ib-vWF and collagen interaction and
eventually assists in a stronger binding between platelets and
collagen via the IIb-IIIa-vWF and collagen interaction. It also
acts as a bridge between the glycoprotein IIb/IIIa receptors to
promote platelet aggregation.
Longnecker DE, Newman MF, Brown DL, Zapol WM.
Anesthesiology. 2nd ed. New York: McGraw-Hill; 2012: 209.
What are the signs and symptoms of von Willebrand
disease?
There are three major forms of the disease that range from mild
to severe, so symptoms may not appear as abruptly or severely
in all patients. However, typical symptoms include easy
bruising, nose bleeds, gastrointestinal bleeding, menorrhagia,
and excessive bleeding during surgical procedures.
Longnecker DE, Newman MF, Brown DL, Zapol WM.
Anesthesiology. 2nd ed. New York: McGraw-Hill; 2012: 209.
How is von Willebrand disease treated?
The most common treatment is desmopressin 0.3 mcg/kg IV in
50 mL of saline over 20 minutes. It may also be administered
as a nasal spray. Other treatment options include vWF
concentrates, alpha-aminocaproic acid, and tranexamic acid.
Longnecker DE, Newman MF, Brown DL, Zapol WM.
Anesthesiology. 2nd ed. New York: McGraw-Hill; 2012: 209-
210.
Besides desmopressin and vWF concentrate, what
is another treatment option for a patient suffering
from von Willebrand disease?
Cryoprecipitate may be administered as it contains
concentrated fibrinogen, fibronectin, vWF, and factors VIII and
XIII. It should be noted, however, that commercially prepared
concentrates of factor VIII-vWF are preferred as cryoprecipitate
still carries a risk of transfusion-transmitted infection.
Hines RL, Marschall KE. Anesthesia & Co-Existing Diseases.
6th ed. Philadelphia, PA: Elsevier-Saunders; 2012: 429.
What side effects of DDAVP administration in a
patient with von Willebrand disease could you expect
to see?
Too rapid administration of DDAVP IV can cause
lightheadedness, flushing, headache, and nausea. It also
possesses a mild antidiuretic effect and can result in water
intoxication in patients receiving multiple DDAVP treatments or
large volumes of fluids.
Hines RL, Marschall KE. Anesthesia & Co-Existing Diseases.
6th ed. Philadelphia, PA: Elsevier-Saunders; 2012: 429.
How are coagulation assays affected in patients with
von Willebrand disease?
They will display a low von Willebrand factor concentration (the
normal is 5-10 mg/dL), a prolonged bleeding time, prolonged
PTT, and a decreased factor VIII activity.
Hines RL, Marschall KE. Anesthesia & Co-Existing Diseases.
6th ed. Philadelphia, PA: Elsevier-Saunders; 2012: 428.
Besides the hereditary form of von Willebrand
disease, what other ways might it appear?
Acquired von Willebrand disease can occur in patients with
some tumors that absorb von Willebrand factor into the tumor
surface.
Butterworth JF, Mackey DC, Wasnick JD. Morgan & Mikhail’s
Clinical Anesthesiology. 5th ed. New York, NY: McGraw-Hill;
2013: 705.
What anesthetic implications should you consider in
the pregnant patient with von Willebrand disease?
When considering anesthetic type, it is worth considering the
increased risk for a spinal or epidural hematoma when
performing neuraxial anesthesia. Normally, vWF factors
increase two or three-fold during the second and third trimester,
which afford a degree of protection from the risk of bleeding, but
this should be verified with laboratory testing if possible. Also,
intranasal desmopressin is safe for use in pregnant patients.
Some individuals, however, have a poor response to DDAVP
and should be treated with vWF concentrates.
Longnecker DE, Newman MF, Brown DL, Zapol WM.
Anesthesiology. 2nd ed. New York: McGraw-Hill; 2012: 210.
What drugs should be avoided in the patient with von
Willebrand disease?
No specific anesthetic agents are contraindicated in these
patients, however, it should be noted that the administration of
NSAIDs such as ketorolac can adversely affect an already
impaired platelet function and result in excessive or
spontaneous bleeding.
Longnecker DE, Newman MF, Brown DL, Zapol WM.
Anesthesiology. 2nd ed. New York: McGraw-Hill; 2012: 209-
210.
