July 2026 QQ Summary: VWD Therapeutic

Our July 2026 QQ asked: What VWD therapeutic is NOT a replacement factor? Our 24 participants responded…

1. Desmopressin: 18 (75%)
2. Humate P: 1 (4%)
3. Vonvendi: 4 (17%)
4. Wilate: 1 (4%)

VWD bleeding is first managed by applying “PRICE:” protection, rest, ice, compression, and elevation. Next, desmopressin (DDAVP) is administered via injection, infusion, and, in Europe, inhalation. Desmopressin is a synthetic analogue of vasopressin, the hormone that controls osmotic balance, blood pressure, and kidney function and reduces urine output. Desmopressin induces VWF secretion from the endothelium and is temporarily effective in VWD type 1 and in some subtypes of VWD type 2. It is ineffective in VWD type 3 and contraindicated for VWD type 2B therapy, a “gain of function” mutant VWF form.  Desmopressin becomes ineffective after 2–3 days because it depletes the natural VWF supply, a phenomenon called “tachyphylaxis.” Prolonged desmopressin therapy reduces plasma sodium, leading to seizures.

The lysine analogues ε-aminocaproic acid (EACA) and tranexamic acid (TXA) are antifibrinolytics that may be used in conjunction with desmopressin, and estrogen therapy is used in males and predominantly in females.

Humate-P, Alphanate (~1990), and Wilate (2009) are human plasma-derived FVIII/VWF concentrates. Vonvendi is a synthetic VWF concentrate that provides no FVIII. CRYO, once the VWD mainstay, is seldom used because of the risk of viral transmission and TACO.

We posted this question to commemorate the 1926 publication by Finnish physician Eric von Willebrand, Von Willebrand, E A (1926). “Hereditär pseudohemofili”. Finska Läkaresällskapets Handlingar (in Swedish). 68: 87–112. Dr. von Willebrand’s title, “Hereditary Pseudohemophilia,” was updated to our current name, von Willebrand disease, in the late 1930s, and the responsible protein was named von Willebrand factor in 1971.

For more VWD information, please navigate to the three-part series “A Coag Conversation, 100 Years of VWD and Upcoming Advances” featuring Robert Sidonio, MD, MSc, Medical Director, Clinical Research Office, Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, and Professor of Pediatrics, Emory University School of Medicine. Also click here to participate in our FF Educational Module on von Willebrand disease.

Sarkar MK, Fritsma GA. Hemorrhagic Disorders and Laboratory Assessment. Chapter 36 of Keohane EM, Butina MM, Mirza KM, Walenga JM. Rodak’s Hematology, Clinical Principles and Applications, Seventh Edition, 2025. Elsevier.