ICD-10 Code D67: Hereditary factor IX deficiency (Hemophilia B)

Key Takeaways

ICD-10 Code D67 is the billable diagnosis code for hereditary factor IX deficiency, also known as Hemophilia B or Christmas disease.

D67 is valid for the 2026 ICD-10-CM fiscal year and falls under block D65-D69 (coagulation defects) within chapter D50-D89 (diseases of the blood).

Coders must distinguish D67 (factor IX deficiency) from D66 (factor VIII deficiency / Hemophilia A) – using the wrong code is a common denial trigger.

Pabau’s claims management software supports accurate ICD-10 code capture, reducing coding errors and claim rejections for haematology and coagulation disorder practices.

ICD-10 Code D67 is assigned for hereditary factor IX deficiency, a genetic bleeding disorder caused by insufficient or absent levels of clotting factor IX in the blood. Factor IX is a serine protease that plays a critical role in the intrinsic coagulation cascade. Without adequate factor IX activity, fibrin clot formation is impaired, leading to prolonged or spontaneous bleeding episodes.

The condition is X-linked recessive and affects primarily males, with females typically serving as carriers. Severity ranges from mild (factor IX activity 6-49%) to moderate (1-5%) to severe (less than 1%), with severe cases most likely to present with spontaneous hemarthroses and deep tissue bleeds. Accurate documentation of the severity and clinical manifestations strengthens the medical record and supports claims management workflows when submitting for factor replacement therapy.

The WHO ICD-10 browser classifies D67 as a single non-subdivided code, meaning there are no additional character extensions. All documented severity levels and clinical variants of hereditary factor IX deficiency map to D67.

Code classification and hierarchy

Understanding where ICD-10 Code D67 sits in the classification hierarchy helps coders navigate adjacent codes and apply correct sequencing rules.

Within the D65-D69 block, D67 sits between D66 (hereditary factor VIII deficiency / Hemophilia A) and D68 (other coagulation defects). The CDC/NCHS ICD-10-CM web tool confirms D67 is valid for fiscal year 2026 with no pending revisions or retirements. Coders can verify billable status annually before claim submission.

Billable status and inclusion terms for D67

ICD-10 Code D67 is a billable diagnosis code and can be submitted directly on insurance claims. It carries four official inclusion terms under the ICD-10-CM tabular list, all of which map to D67 regardless of how the condition is documented in the chart.

• Christmas disease – the eponymous name derived from Stephen Christmas, the first patient described with Hemophilia B in 1952
• Factor IX deficiency (with functional defect) – captures quantitative and qualitative factor IX defects
• Hemophilia B – the most commonly used clinical term in haematology practice
• Plasma thromboplastin component (PTC) deficiency – older terminology still encountered in legacy records

When a physician documents any of these terms in the medical record, the coder assigns D67. No additional specificity characters are available or required. The AAPC Codify ICD-10-CM lookup lists all four inclusion terms under D67 and can be used for cross-referencing during code selection. Accurate code assignment at the encounter level supports compliant patient records and reduces downstream claim edits.

Related codes and the D66 vs D67 distinction

The most critical distinction in hereditary hemophilia coding is between D66 and ICD-10 Code D67. Using these codes interchangeably is incorrect and will trigger clinical documentation queries from payers.

• D66 – Hereditary factor VIII deficiency (Hemophilia A): caused by deficient or defective clotting factor VIII. Hemophilia A accounts for roughly 80% of hemophilia cases and is a separate genetic condition from Hemophilia B
• D67 – Hereditary factor IX deficiency (Hemophilia B): caused by deficient or defective clotting factor IX. Represents approximately 15-20% of hemophilia cases
• D68.0 – Von Willebrand disease: a distinct platelet adhesion disorder involving Von Willebrand factor, not a factor VIII or IX deficiency per se
• D68 – Other coagulation defects: the parent category for less common factor deficiencies (factors I, II, V, VII, X, XI, XII, XIII) not covered by D66 or D67
• M36.2 – Hemophilic arthropathy: a manifestation code assigned for joint damage resulting from recurrent hemarthroses in hemophilia patients. Assign as an additional code alongside D67 when documented

The distinction between D66 and D67 is clinically meaningful because treatment protocols differ. Factor VIII replacement products are used for Hemophilia A; factor IX replacement products are used for Hemophilia B. Payer systems flag mismatches between the diagnosis code and the administered treatment code, which delays reimbursement. Maintaining a compliance management workflow that cross-checks diagnosis against procedure codes before submission prevents these errors. For the full ICD-10-CM code hierarchy, the CMS ICD-10 codes page provides annual updates and official tabular files.

Reduce ICD-10 coding errors and claim rejections

Pabau's claims management software captures diagnosis codes at the point of care, cross-checks against procedure codes, and streamlines billing workflows for haematology and specialist practices.

Book a demo

Documentation requirements for ICD-10 Code D67

Accurate coding of ICD-10 Code D67 depends on what the physician or haematologist documents in the clinical record. The code cannot be assigned from laboratory values alone. The provider must explicitly document one of the recognized clinical terms (hereditary factor IX deficiency, Hemophilia B, Christmas disease, or equivalent).

Key documentation elements that support D67 coding and payer review include:

• Confirmed diagnosis statement – e.g. “Hemophilia B, severe” or “hereditary factor IX deficiency with functional defect” in the assessment or problem list
• Factor IX activity level – percentage documented in the laboratory results section, used to support severity and treatment necessity
• Bleeding history – documentation of hemarthroses, soft tissue bleeds, or prior bleeding episodes establishes clinical context
• Treatment administered – factor IX concentrate or recombinant product name, dose, and route included in the visit note
• Manifestation codes – if hemophilic arthropathy (M36.2) or other complications are present, the provider must document those conditions explicitly for additional codes to be assigned

According to ICD List, D67 has no excludes notes or use-additional-code directives beyond standard coagulation defect coding conventions. However, coders should follow the ICD-10-CM Official Guidelines for Coding and Reporting when sequencing D67 with manifestation codes. Using digital intake forms that capture bleeding history, current medications, and prior factor replacement products at intake reduces documentation gaps that cause coding uncertainty later.

Coding workflow and billing guidance for hereditary factor IX deficiency

Haematology and infusion practices managing Hemophilia B patients encounter a predictable set of billing scenarios involving ICD-10 Code D67. The following guidance covers the most common workflow touchpoints.

Sequencing D67 with procedure and administration codes

When a patient attends for a factor IX infusion, D67 functions as the principal diagnosis code. The procedure is typically coded with an infusion administration CPT code (e.g. 96365 for initial intravenous infusion) alongside the relevant HCPCS J-code for the specific factor IX product administered. The diagnosis-to-procedure linkage must be explicit in the claim. Automated billing workflows that link diagnosis codes to standard procedure code templates for Hemophilia B infusions eliminate manual sequencing errors at submission.

Prior authorisation considerations

Factor IX replacement products are high-cost biologics. Most commercial payers and Medicare Advantage plans require prior authorisation before dispensing. The authorisation request must reference D67 as the documented diagnosis, supported by factor IX activity levels and a haematologist’s attestation of severity. Practices using prescription management software to track factor IX product prescriptions, dosing, and renewal dates are better positioned to respond to payer requests quickly. Prior auth denials most commonly occur when the diagnosis code on the request does not match the code on the dispensing claim, reinforcing the need for consistent D67 assignment across all documentation touchpoints.

ICD-9-CM to ICD-10-CM transition notes

The ICD-9-CM predecessor to D67 is code 286.1 (congenital factor IX disorder). For practices reviewing legacy records or submitting late claims on pre-October 2015 service dates, 286.1 applies. For all current-year encounters, D67 is the correct code. Using AI-assisted clinical documentation that flags outdated codes during note review helps practices avoid submitting retired ICD-9 codes on current claims. The crosswalk mapping is confirmed in the CMS ICD-10 annual update files.

HIPAA compliance and diagnosis code requirements

Under HIPAA transaction and code set standards, electronic claims submitted to payers must use valid ICD-10-CM codes. D67 meets this requirement for the 2026 fiscal year. Practices must ensure their clinic software is HIPAA-compliant in how it stores, transmits, and populates diagnosis codes on claims forms. Any system that allows free-text diagnosis entry without ICD-10 code validation introduces compliance risk for coagulation disorder specialties where code precision directly affects reimbursement for expensive treatments.

Conclusion

Coding hereditary factor IX deficiency accurately depends on one specific code: D67. Confusing it with D66 (Hemophilia A) or the broader D68 category results in claim rejections, prior authorisation mismatches, and documentation queries that slow payment cycles for high-cost biologics. The four inclusion terms, the ICD-9-CM crosswalk from 286.1, and the sequencing rules with M36.2 for hemophilic arthropathy are the core knowledge points every coder handling haematology billing needs.

Pabau’s claims management software captures diagnosis codes at the point of care and supports accurate code linkage across complex multi-code encounters. To see how Pabau handles coding workflows for specialist practices, book a demo.

Continue your research

Need a reference for other blood disorder ICD-10 codes? Intraparenchymal hemorrhage ICD-10 codes covers hemorrhagic condition coding for neurological presentations alongside D65-D69 coagulation codes.

Managing HIPAA-compliant records for specialist patients? HIPAA compliance for medical offices outlines the documentation and data security standards that apply to specialty practice billing.

Looking for guidance on structuring clinical notes for billing? Safer clinical notes provides practical frameworks for documenting diagnoses in ways that support clean claims submission.

Frequently Asked Questions