Overview
Congenital prekallikrein deficiency, also known as Fletcher factor deficiency, is a rare inherited blood disorder. It affects a protein in the blood called prekallikrein (sometimes called Fletcher factor), which plays a role in the contact activation pathway — a system that helps blood clot and also affects inflammation and other body processes. When this protein is missing or not working properly, certain lab tests that measure clotting time come back abnormal, which can be alarming at first. Despite the abnormal lab results, most people with congenital prekallikrein deficiency do not have serious bleeding problems in everyday life. This is one of the most important things to understand about this condition: unlike many other clotting disorders, it usually does not cause spontaneous bleeding or excessive bleeding after injury. The condition is often discovered by accident when routine blood tests are done before surgery or for another reason. Because most people with this condition live normal lives without symptoms, treatment is rarely needed. However, it is important for affected individuals to inform their doctors and surgeons before any procedures, so that the care team is aware of the abnormal clotting test results and can plan accordingly. Regular follow-up with a hematologist (blood specialist) is recommended to monitor the condition and provide guidance.
Key symptoms:
Abnormal blood clotting test results (prolonged aPTT) found on routine lab workUsually no spontaneous bleeding episodesRarely, mild bruisingRarely, mild bleeding after surgery or dental proceduresNo joint bleeds (unlike hemophilia)No internal bleeding in most cases
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Congenital prekallikrein deficiency.
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Rare Disease Specialist
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 prekallikrein deficiency.
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Questions for your doctor
Bring these to your next appointment
- Q1.Does my prekallikrein level put me at any real risk of bleeding during surgery or dental procedures?,Should I carry a medical alert card or letter explaining my diagnosis?,Do my close family members need to be tested for this condition?,How often should I have follow-up blood tests or specialist visits?,Are there any medications I should avoid that might increase my bleeding risk?,What should I tell my surgeon or dentist before a procedure?,Is there any new research or treatment being developed for this condition?
Common questions about Congenital prekallikrein deficiency
What is Congenital prekallikrein deficiency?
Congenital prekallikrein deficiency, also known as Fletcher factor deficiency, is a rare inherited blood disorder. It affects a protein in the blood called prekallikrein (sometimes called Fletcher factor), which plays a role in the contact activation pathway — a system that helps blood clot and also affects inflammation and other body processes. When this protein is missing or not working properly, certain lab tests that measure clotting time come back abnormal, which can be alarming at first. Despite the abnormal lab results, most people with congenital prekallikrein deficiency do not have s
How is Congenital prekallikrein deficiency inherited?
Congenital prekallikrein deficiency follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Congenital prekallikrein deficiency?
1 specialists and care centers treating Congenital prekallikrein deficiency are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.