Overview
Congenital factor XI deficiency, also known as hemophilia C or Rosenthal syndrome, is a rare inherited bleeding disorder. It is caused by low levels or poor function of factor XI, a protein in the blood that helps form clots to stop bleeding. Unlike other types of hemophilia, the severity of bleeding in factor XI deficiency does not always match how low the factor level is, which can make it unpredictable. People with this condition may experience prolonged bleeding after surgery, dental procedures, or trauma. Women may have heavy or prolonged menstrual periods and bleeding after childbirth. Spontaneous bleeding into joints or muscles, which is common in hemophilia A and B, is rare in factor XI deficiency. Many people with mild deficiency may not know they have the condition until they face a surgical challenge or injury. Treatment focuses on preventing and managing bleeding episodes, especially around surgeries or procedures. Options include fresh frozen plasma, factor XI concentrates (where available), and antifibrinolytic drugs like tranexamic acid. There is no cure, but with proper planning and management, most people with factor XI deficiency live normal, healthy lives.
Also known as:
Key symptoms:
Prolonged bleeding after surgery or dental workHeavy or prolonged menstrual periodsExcessive bleeding after childbirthProlonged bleeding after injury or traumaEasy bruisingNosebleeds that are hard to stopBleeding from the gumsBlood in the urineProlonged bleeding after circumcisionBleeding after tonsil removal or other throat surgery
Clinical phenotype terms (9)— hover any for plain English
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
1 eventCOAGADEX: FDA approved
COAGADEX is indicated in adults and children with hereditary Factor X deficiency for routine prophylaxis to reduce the frequency of bleeding episodes; on-demand treatment and control of bleeding episodes; and perioperative management of bleeding in patients with mild and moderate hereditary Factor X deficiency.
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableCOAGADEX
COAGADEX is indicated in adults and children with hereditary Factor X deficiency for routine prophylaxis to reduce the frequency of bleeding episodes; on-demand treatment and control of bleeding episo…
COAGADEX is indicated in adults and children with hereditary Factor X deficiency for routine prophylaxis to reduce the frequency of bleeding episodes; on-demand treatment and control of bleeding episodes; and perioperative management of bleeding in patients with mild and moderate hereditary Factor X deficiency.
Clinical Trials
View all trials with filters →No actively recruiting trials found for Congenital factor XI deficiency at this time.
New trials open frequently. Follow this disease to get notified.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Congenital factor XI deficiency.
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Caregiver Resources
NORD Caregiver Resources
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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.What is my (or my child's) factor XI level, and what does that mean for bleeding risk?,What precautions should I take before surgery, dental work, or other procedures?,Should I carry a medical alert card or bracelet?,Are there any medications I should avoid, such as aspirin or blood thinners?,What treatment options are available in my area if I have a bleeding episode?,Should my family members be tested for factor XI deficiency?,If I am a woman, how should I manage heavy periods or plan for pregnancy and childbirth?
Common questions about Congenital factor XI deficiency
What is Congenital factor XI deficiency?
Congenital factor XI deficiency, also known as hemophilia C or Rosenthal syndrome, is a rare inherited bleeding disorder. It is caused by low levels or poor function of factor XI, a protein in the blood that helps form clots to stop bleeding. Unlike other types of hemophilia, the severity of bleeding in factor XI deficiency does not always match how low the factor level is, which can make it unpredictable. People with this condition may experience prolonged bleeding after surgery, dental procedures, or trauma. Women may have heavy or prolonged menstrual periods and bleeding after childbirth.
Which specialists treat Congenital factor XI deficiency?
3 specialists and care centers treating Congenital factor XI deficiency are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Congenital factor XI deficiency?
1 patient support program are currently tracked on UniteRare for Congenital factor XI deficiency. See the treatments and support programs sections for copay assistance, eligibility, and contact details.