Short answer · Medically reviewed summary · Last updated: 2026-04-07

The ICD-10 codes for Haemophilia are D66 for Haemophilia A (factor VIII deficiency) and D67 for Haemophilia B (factor IX deficiency), while the older ICD-9 codes are 286.0 for Haemophilia A and 286.1 for Haemophilia B. These diagnostic codes are essential for healthcare providers to accurately document and bill for the specialized care required for individuals living with this bleeding disorder. What is the clinical significance of these Haemophilia codes? In clinical practice, identifying the correct code for Haemophilia is critical for ensuring patients receive appropriate access to clotting factor concentrates and comprehensive care.

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ICD10 code of Haemophilia and ICD9 code

ICD-10 and ICD-9 codes for Haemophilia, with classification details for clinicians, coders and patients.

ICD9 and ICD10 codes of Haemophilia

The ICD-10 codes for Haemophilia are D66 for Haemophilia A (factor VIII deficiency) and D67 for Haemophilia B (factor IX deficiency), while the older ICD-9 codes are 286.0 for Haemophilia A and 286.1 for Haemophilia B. These diagnostic codes are essential for healthcare providers to accurately document and bill for the specialized care required for individuals living with this bleeding disorder.



What is the clinical significance of these Haemophilia codes?


In clinical practice, identifying the correct code for Haemophilia is critical for ensuring patients receive appropriate access to clotting factor concentrates and comprehensive care. Haemophilia A and B are X-linked recessive genetic disorders, meaning they primarily affect males. Because the severity of Haemophilia—classified as mild, moderate, or severe based on the baseline clotting factor activity—dictates the frequency of bleeding episodes, these ICD codes help track patient outcomes and treatment efficacy within the healthcare system. At DiseaseMaps.org, we have seen 334 people with Haemophilia join our community, highlighting the importance of standardized medical coding in connecting patients to specialized treatment centers.



How are Haemophilia A and B distinguished in medical coding?


While both conditions share similar clinical presentations, such as spontaneous joint bleeding (hemarthrosis) and prolonged bleeding after injury, they are caused by deficiencies in different clotting proteins. Medical coding distinguishes between these types to ensure the correct therapeutic approach, as the treatment for Haemophilia A involves factor VIII replacement, whereas Haemophilia B requires factor IX replacement. The following list summarizes the diagnostic classification:



  • Haemophilia A (ICD-10: D66): Deficiency of clotting factor VIII, affecting approximately 1 in 5,000 to 10,000 males.

  • Haemophilia B (ICD-10: D67): Deficiency of clotting factor IX, also known as Christmas disease, occurring in approximately 1 in 25,000 to 30,000 males.

  • Acquired Haemophilia (ICD-10: D68.31): A rare condition where the body develops antibodies against clotting factors, distinct from the congenital forms.



Is Haemophilia diagnosis strictly based on these codes?


No, an ICD code is merely a billing and administrative tool used for medical records. A formal diagnosis of Haemophilia is established through specialized coagulation studies, including a complete blood count (CBC), prothrombin time (PT), activated partial thromboplastin time (aPTT), and specific factor activity assays. Genetic testing is also performed to identify the specific mutation in the F8 or F9 gene, which is vital for family planning and carrier testing for relatives of those with Haemophilia.



How can patients manage the emotional impact of a chronic diagnosis?


Living with a lifelong bleeding disorder can feel isolating. Beyond the physical management of factor levels, it is important to address the psychological burden. Chronic illness often brings anxiety regarding potential injuries or the stress of managing frequent infusions. Connecting with others in the Haemophilia community can provide invaluable peer support and shared coping strategies, reminding you that you are not navigating this journey alone.



Next steps



  • Consult a hematologist at a designated Haemophilia Treatment Center (HTC) to ensure your medical records accurately reflect your specific factor deficiency.

  • Request a copy of your genetic report to understand your specific mutation, which can be useful for future family planning.

  • Join the DiseaseMaps.org community to share experiences and learn from 334 other members living with this condition.

  • Carry a medical alert bracelet or digital identification card that lists your specific diagnosis and emergency contact information.



Medical disclaimer: This content is for informational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician or qualified health provider with any questions regarding a medical condition.



References



  • World Federation of Hemophilia (WFH): wfh.org

  • NIH Genetic and Rare Diseases Information Center (GARD): rarediseases.info.nih.gov

  • Orphanet (Portal for rare diseases and orphan drugs): orpha.net

  • OMIM (Online Mendelian Inheritance in Man): omim.org

Author: DiseaseMaps Editorial Team
Reviewed against authoritative medical sources (NIH GARD, Orphanet, OMIM)
Last updated: 2026-04-07
Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
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