To many people, bleeding disorders are alike — but von Willebrand disease and hemophilia are not the same, and understanding the difference can shape diagnosis, treatment, and long-term care.
“Von Willebrand disease is the most common bleeding disorder, and it can affect up to 1% of the population,” which is about 1 in 100 people, says Magdalena Lewandowska, M.D., adult hematologist and associate medical director at the Indiana Hemophilia & Thrombosis Center. “Hemophilia A affects about 1 in 5,000 individuals. Hemophilia B is rarer, affecting about 1 in 20,000.”
Here's a closer look at von Willebrand disease (VWD) and hemophilia and why it’s important to know more about them.
Causes and Inheritance Patterns
VWD and hemophilia both affect the body’s ability to clot, but they do so in different ways.
VWD occurs when the body does not make enough von Willebrand factor, a protein that helps platelets to stick together and clotting factor VIII to stabilize. Hemophilia, on the other hand, occurs when the body does not make enough factor VIII (hemophilia A) or factor IX (hemophilia B).
How VWD and hemophilia are inherited also differ.
Most instances of VWD are autosomal dominant, meaning a child inherits the condition by getting one copy of the affected gene. “You have a 50-50 chance of getting the gene from your mom or dad, regardless of your gender,” Lewandowska says.
Because of this, providers often find that VWD appears across multiple generations. Families may notice grandparents, parents, and children with similar bleeding symptoms.
Hemophilia is inherited via the X chromosome, and since males only have one of those chromosomes (they have XY), they are more likely than females (who have XX) to have the bleeding disorder. As Lewandowska points out, however, females can also inherit the gene, and about a third of them have factor levels low enough to cause bleeding symptoms.
Inheritance scenarios can be complex, which is why understanding the diagnosis of VWD and hemophilia — plus other inherited blood and bleeding disorders — matters for individuals and families.
von Willebrand Disease Symptoms vs. Hemophilia Bleeding Patterns & Diagnosis
One of the most noticeable differences between VWD and hemophilia is in bleeding.
With VWD, “the story is often there are nosebleeds,” Lewandowska says, but she acknowledges those can be wrongfully assumed to be normal, particularly in childhood. “Another typical symptom is easy bruising.”
During surgeries and dental procedures, bleeding can be difficult to stop, which is another potential indicator of von Willebrand disease.
Also, young girls and women are often diagnosed after going to a hemophilia treatment center for heavy menstrual bleeding.
Hemophilia, by contrast, often involves deeper bleeding.
“With hemophilia, especially in severe cases, we see spontaneous bleeding events,” Lewandowska says. “There is bleeding into joints and muscles. Little boys will bleed with circumcision. There’s a much higher risk of spontaneous head bleeds in babies.” Mild hemophilia can also lead to bleeds from overuse or minor injuries, she adds.
There can be overlap in the two conditions — particularly in rare, severe forms of VWD — but in general, bleeding patterns help guide diagnosis.
How to Treat von Willebrand Disease and Hemophilia
Even though clotting factor levels in VWD and hemophilia may look similar in blood tests, “it is different with the way that people bleed and the way that we manage these patients,” Lewandowska says.
For many people with VWD, treatment may only be needed before surgeries or procedures, or after injuries.
“To get them through a procedure or if they’re having bleeding symptoms, we often use desmopressin, or DDAVP, to allow them to release more von Willebrand factor and factor VIII from where they’re stored in endothelial cells,” she explains.
DDAVP also works well for many people with mild hemophilia A, she adds. But more severe cases of hemophilia often need more intensive management.
“People with severe hemophilia will require prophylaxis, and that’s really important to prevent joint damage, which can then subsequently impact their quality of life,” Lewandowska says. Prophylaxis is a preventive treatment delivered intravenously (into a vein) or subcutaneously (under the skin) one to three times a week to help reduce spontaneous bleeding and protect long-term joint health.
When people with hemophilia and severe VWD regularly seek comprehensive care, it gives providers the opportunity to watch for the development of inhibitors, which are antibodies that make standard treatments ineffective.
How Daily Life Can Look
What does daily life look like for someone who has VWD or hemophilia?
“For type 1 von Willebrand disease, we ask all of our patients to call with any injuries, bleeds, surgeries, or procedures — but in between bleeding challenges, people usually have a regular day-to-day life,” Lewandowska says.
“There are specific issues we need to address, such as nosebleeds, or if there is any vaginal bleeding when a woman is pregnant,” she adds.
“Hemophilia impacts people a lot more,” she explains. “We’re seeing them more intensely, with closer follow-up in an HTC. We’re checking blood tests regularly to rule out the development of an inhibitor.”
Proactive treatment with prophylaxis can head off joint disease.
Why Knowing the Differences Matters
Whether someone has VWD or hemophilia, getting the right diagnosis and receiving education on how to successfully manage the condition are key.
“It’s important for people with suspected bleeding disorders to be evaluated at a hemophilia treatment center, where they encounter physicians and staff who deal with this all the time,” Lewandowska says.
“My job is really to prevent people from having issues and to live their best lives aside from the bleeding disorder,” she continues. “It depends on their bleeding history, the gene they inherited, and their goals, needs, and desires for maintaining an active lifestyle.”