Understanding Hemophilia C (Factor XI Deficiency)
Hemophilia C, also known as Factor XI deficiency or Rosenthal syndrome, is a rare inherited bleeding disorder that usually causes milder bleeding than Hemophilia A or B.
- Autosomal recessive inheritance
- Factor XI deficiency
- Usually mild bleeding tendency
- Most Common In
- Both sexes (autosomal recessive)
- Notable Population
- Higher carrier rate in Ashkenazi Jewish populations
- Deficient Factor
- Factor XI
- Advanced Therapies
- Factor XI concentrate, FFP, novel agents
Condition Overview
Hemophilia C, caused by mutations in the F11 gene, results in deficient or dysfunctional Factor XI, a protein involved in the blood clotting cascade. Unlike Hemophilia A and B, it follows an autosomal recessive inheritance pattern and affects males and females roughly equally. Bleeding tendency is generally milder and most often triggered by surgery, dental work, or trauma rather than occurring spontaneously.
Types and Classification
Hemophilia C severity depends on whether one or both copies of the F11 gene are affected.
Symptoms and Signs
Many individuals with Hemophilia C are asymptomatic until they undergo surgery, dental extraction, or experience significant trauma.
Causes and Risk Factors
Hemophilia C is an inherited condition caused by mutations affecting the F11 gene.
Diagnosis and Investigations
Diagnosis often follows unexpected bleeding after surgery or is identified through routine pre-operative screening.
Severity Classification
Hemophilia C severity is generally classified by Factor XI activity level and corresponding bleeding risk.
Standard Treatment
Treatment is typically reserved for surgical, dental, or trauma situations rather than ongoing prophylaxis, since spontaneous bleeding is uncommon.
Advanced & Emerging Therapies
Therapeutic options for Hemophilia C remain more limited than for Hemophilia A or B, with research ongoing.
Antifibrinolytic Agents
Tranexamic acid
Helps stabilize clots and reduce bleeding, particularly for mucosal bleeding and minor procedures.
Factor Replacement
Factor XI concentrate
Available in some regions for perioperative management of severe deficiency; carries a rare risk of thrombosis.
Recombinant Factor Approaches
Investigational recombinant Factor XI products
Being studied to provide safer, more widely available replacement therapy.
Biomarkers & Monitoring
Laboratory markers guide diagnosis and perioperative risk assessment.
When to Seek a Second Opinion
A second opinion can help with perioperative planning and clarifying bleeding risk in less typical cases.
Clinical Trials & Research
Prognosis & Outcomes
Most people with Hemophilia C have a good long-term outlook, with bleeding risk mainly relevant around surgery, dental work, or childbirth rather than day-to-day life.
Supportive Care
Supportive measures focus on prevention and preparedness around procedures and major life events.
How CancerFax Helps You Explore Treatment Options
CancerFax helps patients with Hemophilia C access specialist hematology review and coordinate perioperative bleeding management plans with experienced centers.
Get a free case reviewFrequently Asked Questions
Hemophilia C is a rare inherited bleeding disorder caused by deficiency of Factor XI, generally causing milder bleeding than Hemophilia A or B.
Yes, because it is inherited in an autosomal recessive pattern rather than being X-linked.
No, it involves a deficiency of a different clotting factor (Factor XI) and typically causes milder, procedure-related bleeding.
Diagnosis involves an aPTT screening test followed by a specific Factor XI activity assay.
Certain founder mutations are more frequent in individuals of Ashkenazi Jewish ancestry, raising carrier rates in that population.
Most patients only need treatment around surgery, dental work, or childbirth rather than ongoing daily therapy.
Options include antifibrinolytic medications, fresh frozen plasma, and Factor XI concentrate where available.
Yes, it can increase bleeding risk during delivery, so coordinated obstetric and hematology planning is important.
There is no cure, but most patients manage well with planned precautions around procedures and major life events.
Yes. CancerFax assists with medical report review, second opinion coordination, and connecting patients with specialist centers for perioperative bleeding management and international coordination where relevant.
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