Short answer · Medically reviewed summary · Last updated: 2026-05-08
Factor XIII Deficiency is classified under the ICD-10-CM code D68.2 (Hereditary deficiency of other clotting factors) and the ICD-9-CM code 286.59 (Other hemorrhagic conditions). These codes are used by medical professionals to document this rare bleeding disorder in clinical records and insurance claims. What is the clinical significance of Factor XIII Deficiency? Factor XIII Deficiency is a rare, life-threatening coagulation disorder that prevents the formation of stable blood clots.
ICD10 code of Factor XIII Deficiency and ICD9 code
ICD-10 and ICD-9 codes for Factor XIII Deficiency, with classification details for clinicians, coders and patients.
Factor XIII Deficiency is classified under the ICD-10-CM code D68.2 (Hereditary deficiency of other clotting factors) and the ICD-9-CM code 286.59 (Other hemorrhagic conditions). These codes are used by medical professionals to document this rare bleeding disorder in clinical records and insurance claims.
What is the clinical significance of Factor XIII Deficiency?
Factor XIII Deficiency is a rare, life-threatening coagulation disorder that prevents the formation of stable blood clots. Unlike hemophilia, which involves a failure to form a clot, individuals with Factor XIII Deficiency form a weak clot that dissolves prematurely, leading to delayed, severe bleeding. The condition affects approximately 1 in 1 to 2 million people worldwide, making it one of the rarest clotting factor deficiencies known to medicine.
How is Factor XIII Deficiency diagnosed and coded?
Diagnosis of Factor XIII Deficiency typically involves specialized laboratory testing, as standard coagulation screens like PT and PTT often return normal results. Clinicians use specific Factor XIII activity assays to confirm the diagnosis. When coding for this condition, providers use D68.2 in the ICD-10 system to signify a deficiency in the fibrin-stabilizing factor. Accurate coding is essential for patients in our DiseaseMaps.org community, as it ensures proper documentation for specialized therapies such as prophylactic factor replacement.
What are the common clinical presentations?
Patients living with Factor XIII Deficiency often experience specific, hallmark symptoms that distinguish the condition from other coagulopathies:
- Umbilical cord bleeding at birth (a common early indicator).
- Intracranial hemorrhage, which is the leading cause of morbidity.
- Delayed wound healing and spontaneous hematomas.
- Recurrent miscarriages in affected females due to the role of Factor XIII in placental attachment.
Is Factor XIII Deficiency hereditary?
Yes, Factor XIII Deficiency is typically inherited in an autosomal recessive pattern, meaning an individual must inherit two copies of the mutated gene to manifest the disease. Genetic counseling is highly recommended for families planning pregnancies to assess the carrier status of parents.
Next steps
- Consult with a hematologist who specializes in rare bleeding disorders.
- Connect with the 5 members of the DiseaseMaps.org community who are living with Factor XIII Deficiency to share experiences.
- Request a referral to a comprehensive Hemophilia Treatment Center (HTC).
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Factor XIII Deficiency.
- Orphanet: Rare disease database for Factor XIII deficiency (ORPHA:333).
- OMIM (Online Mendelian Inheritance in Man): F13A1 and F13B gene entries.
- World Federation of Hemophilia (WFH) guidelines on rare bleeding disorders.
