Thrombomodulin-related bleeding disorder

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ORPHA:436169OMIM:614486D68.3
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Overview

Thrombomodulin-related bleeding disorder is a very rare inherited condition caused by changes (mutations) in the THBD gene, which provides instructions for making a protein called thrombomodulin. Thrombomodulin plays an important role in controlling blood clotting. It sits on the surface of cells lining blood vessels and helps regulate how quickly and effectively your blood forms clots. When this protein does not work properly, the balance between bleeding and clotting is disrupted, leading to an increased tendency to bleed. People with this disorder may experience prolonged bleeding after injuries, surgery, or dental procedures. They may also have easy bruising, nosebleeds, heavy menstrual periods in women, and in some cases, bleeding into joints or soft tissues. The severity of symptoms can vary from person to person, even within the same family. Treatment is mainly supportive and focused on managing bleeding episodes. There is no cure for this condition. Doctors may use clotting factor replacement therapies, antifibrinolytic medications (such as tranexamic acid), or other blood products to help control bleeding when it occurs. Patients are advised to work closely with a hematologist who specializes in bleeding disorders to develop a personalized management plan.

Also known as:

Key symptoms:

Easy bruisingProlonged bleeding after cuts or injuriesExcessive bleeding after surgery or dental workFrequent or prolonged nosebleedsHeavy menstrual periods in womenBleeding into jointsBleeding into soft tissues or musclesProlonged bleeding after childbirthBlood in urine or stool in some cases

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Thrombomodulin-related bleeding disorder.

View clinical trials →

No actively recruiting trials found for Thrombomodulin-related bleeding disorder at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Thrombomodulin-related bleeding disorder community →

No specialists are currently listed for Thrombomodulin-related bleeding disorder.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Thrombomodulin-related bleeding disorder.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Thrombomodulin-related bleeding disorder

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my (or my child's) bleeding tendency based on current test results?,What medications should I always have on hand in case of a bleeding episode?,Are there any activities or sports I should avoid?,What should I tell my dentist or surgeon before a procedure?,Should other family members be tested for this condition?,What are the warning signs that I need to go to the emergency room?,Are there any clinical trials or new treatments being studied for this condition?

Common questions about Thrombomodulin-related bleeding disorder

What is Thrombomodulin-related bleeding disorder?

Thrombomodulin-related bleeding disorder is a very rare inherited condition caused by changes (mutations) in the THBD gene, which provides instructions for making a protein called thrombomodulin. Thrombomodulin plays an important role in controlling blood clotting. It sits on the surface of cells lining blood vessels and helps regulate how quickly and effectively your blood forms clots. When this protein does not work properly, the balance between bleeding and clotting is disrupted, leading to an increased tendency to bleed. People with this disorder may experience prolonged bleeding after in

How is Thrombomodulin-related bleeding disorder inherited?

Thrombomodulin-related bleeding disorder follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.