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Congenital factor X deficiency is an inherited bleeding disorder with a decreased antigen and/or activity of factor X (FX) and characterized by mild to severe bleeding symptoms.
Features include always present findings: Prolonged prothrombin time and Reduced factor X activity; and very common findings: Prolonged bleeding after surgery and Prolonged bleeding after dental extraction. 21 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Blood and immune system | 5 | Prolonged bleeding after surgery, Gingival bleeding, Prolonged bleeding after dental extraction |
Heart and blood vessels | 2 | Intracranial hemorrhage, Subarachnoid hemorrhage |
Bones and joints | 1 | Joint hemorrhage |
Kidneys and urinary system | 1 | Blood in the urine (hematuria) |
Digestive system | 1 | Gastrointestinal hemorrhage |
F10 encodes coagulation factor X (488 aa). Factor Xa is a vitamin K-dependent glycoprotein that converts prothrombin to thrombin in the presence of factor Va, calcium and phospholipid during blood clotting. Highest expression in Cervix Ectocervix (75.5 TPM) and Liver (73.4 TPM).
Congenital factor X deficiency is caused by mutations in the F10 gene on chromosome 13.
The F10 protein participates in factor X activation peptide pathway.
F10 is classified as a druggable target (Druggable Genome, Enzyme, External Side Of Plasma Membrane, and Protease categories) with score 3.1.
Genetic testing for F10 is available. Testing is considered confirmatory for diagnosis.
1 FDA-approved treatment is available for congenital factor X deficiency, including Coagulation Factor X (Human) (Coagadex, approved 2015).
Brand Name | Generic Name | Mechanism | Approved | Market Status |
|---|---|---|---|---|
Coagadex | Coagulation Factor X (Human) | — | 2015 | Available |
2 trials found
Phenotype severity distribution: 2 always present features, 2 very common features, 2 common features.
Estimated prevalence: 1-9 in 1,000,000 (Rare).
2 clinical trials registered, 1 recruiting. Interventions under study include other interventions and biologic therapy. Pipeline includes 1 PHASE3. Research is sponsored by a mix of industry and academic institutions.
38 publications have been identified in PubMed for congenital factor X deficiency. Research spans Case Report / Case Series (55%), Review / Meta-Analysis (16%), and Basic Science / Preclinical (13%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 21 | 55% |
Research summaries | 6 | 16% |
Laboratory research | 5 | 13% |
Other research | 4 | 11% |
Clinical study results | 1 | 3% |
Disease patterns and progression | 1 | 3% |
Sbibih Y (2026). [PMID: 42170305](https://pubmed.ncbi.nlm.nih.gov/42170305/). *Leuk Res Rep*. [Case Report / Case Series]
Connell NT (2026). [PMID: 41988968](https://pubmed.ncbi.nlm.nih.gov/41988968/). *Haemophilia*. [Review / Meta-Analysis]
Kumar V (2026). [PMID: 41818439](https://pubmed.ncbi.nlm.nih.gov/41818439/). *Haemophilia*. [Other]
Rossoni C (2026). [PMID: 41923907](https://pubmed.ncbi.nlm.nih.gov/41923907/). *Front Nutr*. [Case Report / Case Series]
Gat R (2026). [PMID: 40159294](https://pubmed.ncbi.nlm.nih.gov/40159294/). *Acta Haematol*. [Case Report / Case Series]
Pokhrel KM (2026). [PMID: 41836993](https://pubmed.ncbi.nlm.nih.gov/41836993/). *Int J Surg Case Rep*. [Case Report / Case Series]
Veizaj D (2026). [PMID: 42176283](https://pubmed.ncbi.nlm.nih.gov/42176283/). *J Chem Inf Model*. [Basic Science / Preclinical]
Kattoush I (2026). [PMID: 42144914](https://pubmed.ncbi.nlm.nih.gov/42144914/). *Blood Coagul Fibrinolysis*. [Case Report / Case Series]
Ferreira VJP (2026). [PMID: 42226715](https://pubmed.ncbi.nlm.nih.gov/42226715/). *Expert Rev Clin Pharmacol*. [Review / Meta-Analysis]
Keskin D (2026). [PMID: 42227468](https://pubmed.ncbi.nlm.nih.gov/42227468/). *Curr Rheumatol Rev*. [Case Report / Case Series]
Data assembled from 9 of 12 sources · Last updated Oct 3, 2026, 11:00 PM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center