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D67 ICD-10-CM Code: Hereditary factor IX deficiency

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
4 inclusion terms
Risk adjustment
CMS-HCC V28: 1 category · CMS-HCC V22 category 46

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43, Appendix B.

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Risk Adjustment (CMS-HCC)

Payment categories this diagnosis maps to under CMS-HCC V28, payment year 2026.

  • HCC 111 — Hemophilia, Male (supersedes HCC 112)

Other models: CMS-HCC V22 HCC 46

Risk scores depend on the enrollee's full accepted diagnosis set and segment; a category mapping alone does not determine payment.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for D67 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Christmas disease
  • Factor IX deficiency (with functional defect)
  • Hemophilia B
  • Plasma thromboplastin component [PTC] deficiency

Code Overview

Hereditary factor IX deficiency is a billable ICD-10-CM diagnosis code (D67).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Indexed Clinical Terms (9)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Official Coding Guidelines

No excerpt in the ICD-10-CM Official Guidelines names this code specifically. Its chapter carries only this chapter-wide note:

Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

Reserved for future guideline expansion

Verify Before Coding

  • MCC as a secondary diagnosis (FY2026). Can raise the stay's MS-DRG severity tier.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder structured relationships — computed from published CMS and AHRQ datasets

Other codes that name D67 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 10 Excludes2 notes: R70 — Elevated erythrocyte sedimentation rate and abnormality of plasma viscosity, R70-R79 — Abnormal findings on examination of blood, without diagnosis (R70-R79), R71 — Abnormality of red blood cells, R73 — Elevated blood glucose level, R74 — Abnormal serum enzyme levels, R75 — Inconclusive laboratory evidence of human immunodeficiency virus [HIV], R76 — Other abnormal immunological findings in serum, R77 — Other abnormalities of plasma proteins, R78 — Findings of drugs and other substances, not normally found in blood, R79 — Other abnormal findings of blood chemistry.

These codes’ tabular lists name this diagnosis as distinct — both may be reported when both are documented.

Referenced by 22 Code First instructions across 2 chapters: M36.2 — Hemophilic arthropathy, T36 — Poisoning by, adverse effect of and underdosing of systemic antibiotics, T36-T39 — Antibiotics & Anti-infectives (T36-T39), T36-T50 — Poisoning by, adverse effects of and underdosing of drugs, medicaments and biological substances (T36-T50), T37 — Poisoning by, adverse effect of and underdosing of other systemic anti-infectives and antiparasitics, T38 — Poisoning by, adverse effect of and underdosing of hormones and their synthetic substitutes and antagonists, not elsewhere classified, T39 — Poisoning by, adverse effect of and underdosing of nonopioid analgesics, antipyretics and antirheumatics, T40 — Poisoning by, adverse effect of and underdosing of narcotics and psychodysleptics [hallucinogens], T40-T41 — Analgesics & Antipyretics (T40-T41), T41 — Poisoning by, adverse effect of and underdosing of anesthetics and therapeutic gases, T42 — Poisoning by, adverse effect of and underdosing of antiepileptic, sedative- hypnotic and antiparkinsonism drugs, T42-T43 — Anticonvulsants & Psychotropics (T42-T43), T43 — Poisoning by, adverse effect of and underdosing of psychotropic drugs, not elsewhere classified, T44 — Poisoning by, adverse effect of and underdosing of drugs primarily affecting the autonomic nervous system, T44-T46 — Cardiovascular & Gastrointestinal (T44-T46), T45 — Poisoning by, adverse effect of and underdosing of primarily systemic and hematological agents, not elsewhere classified, T46 — Poisoning by, adverse effect of and underdosing of agents primarily affecting the cardiovascular system, T47 — Poisoning by, adverse effect of and underdosing of agents primarily affecting the gastrointestinal system, T47-T50 — Hormones & Systemic Agents (T47-T50), T48 — Poisoning by, adverse effect of and underdosing of agents primarily acting on smooth and skeletal muscles and the respiratory system, +2 more.

Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: MCC — Major Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 50 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 1 MS-DRG: DRG 813 (MDC 16).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):BLD006 — Coagulation and hemorrhagic disorders (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical process (MS-DRG)

Acts as MCC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

D47.4 — Osteomyelofibrosis, D65 — Disseminated intravascular coagulation [defibrination syndrome], D66 — Hereditary factor VIII deficiency, D68.00 — Von Willebrand disease, unspecified, D68.01 — Von Willebrand disease, type 1, D68.020 — Von Willebrand disease, type 2A, D68.021 — Von Willebrand disease, type 2B, D68.022 — Von Willebrand disease, type 2M, D68.023 — Von Willebrand disease, type 2N, D68.029 — Von Willebrand disease, type 2, unspecified, D68.03 — Von Willebrand disease, type 3, D68.04 — Acquired von Willebrand disease, D68.09 — Other von Willebrand disease, D68.1 — Hereditary factor XI deficiency, D68.2 — Hereditary deficiency of other clotting factors, D68.311 — Acquired hemophilia, D68.312 — Antiphospholipid antibody with hemorrhagic disorder, D68.318 — Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors, D68.32 — Hemorrhagic disorder due to extrinsic circulating anticoagulants, D68.4 — Acquired coagulation factor deficiency, +29 more

Same CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Hemophilia, Male) for risk-adjusted payment.

D66 — Hereditary factor VIII deficiency

Related risk categories

These categories interact through CMS's HCC hierarchy — one can suppress the other's risk-adjustment weight when both are present on a claim.

Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Coagulation and hemorrhagic disorders).

D47.3 — Essential (hemorrhagic) thrombocythemia, D65 — Disseminated intravascular coagulation [defibrination syndrome], D66 — Hereditary factor VIII deficiency, D68.0 — Von Willebrand disease, D68.00 — Von Willebrand disease, unspecified, D68.01 — Von Willebrand disease, type 1, D68.020 — Von Willebrand disease, type 2A, D68.021 — Von Willebrand disease, type 2B, D68.022 — Von Willebrand disease, type 2M, D68.023 — Von Willebrand disease, type 2N, D68.029 — Von Willebrand disease, type 2, unspecified, D68.03 — Von Willebrand disease, type 3, D68.04 — Acquired von Willebrand disease, D68.09 — Other von Willebrand disease, D68.1 — Hereditary factor XI deficiency, D68.2 — Hereditary deficiency of other clotting factors, D68.311 — Acquired hemophilia, D68.312 — Antiphospholipid antibody with hemorrhagic disorder, D68.318 — Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors, D68.32 — Hemorrhagic disorder due to extrinsic circulating anticoagulants, +31 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Deficiency, deficient”, “Hemophilia”; these codes share that main term but sit in a different category of the Tabular List.

D50.9 — Iron deficiency anemia, unspecified (iron, anemia), D51.0 — Vitamin B12 deficiency anemia due to intrinsic factor deficiency (intrinsic, factor), D51.2 — Transcobalamin II deficiency (transcobalamine II), D53.0 — Protein deficiency anemia (protein, anemia), D55.0 — Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency (glucose-6-phosphate dehydrogenase, anemia), D64.9 — Anemia, unspecified (hemoglobin), D65 — Disseminated intravascular coagulation [defibrination syndrome] (fibrinogen, acquired), D66 — Hereditary factor VIII deficiency, D68.01 — Von Willebrand disease, type 1 (von Willebrand factor, partial quantitative), D68.03 — Von Willebrand disease, type 3 (von Willebrand factor, total quantitative), D68.1 — Hereditary factor XI deficiency (C), D68.2 — Hereditary deficiency of other clotting factors (SPCA), D68.311 — Acquired hemophilia (acquired), D68.4 — Acquired coagulation factor deficiency (calcipriva), D68.59 — Other primary thrombophilia (protein, C), D68.8 — Other specified coagulation defects (factor, multiple), D68.9 — Coagulation defect, unspecified (clotting), D69.19 — Other qualitative platelet defects (platelet NEC), D71.1 — Leukocyte adhesion deficiency (leukocyte adhesion), D74.0 — Congenital methemoglobinemia (NADH diaphorase or reductase), +175 more

Lab tests where this diagnosis supports Medicare coverage (NCD)

Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.

Fecal Occult Blood Test (FOBT), Iron Panel, Partial Thromboplastin Time (PTT) Test, Prothrombin Time Test and INR (PT/INR)

Contextual Map

Every relationship of D67 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run D67 with these 16 related codes in Claim Check

Hierarchy

Referenced by Excludes2 notes (10)

Referenced by Code First instructions (22)

Clinical classification (CCSR)

Risk adjustment (CMS-HCC)

  • HCC 111 — Hemophilia, Male [CMS-HCC]: “Hemophilia, Male — supersedes HCC 112 (Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions)”— CMS-HCC V28 · 2026

MS-DRG Grouper

  • MCC — Major Complication or Comorbidity [MS-DRG severity]: “As a secondary diagnosis this code can raise the stay's MS-DRG severity tier (MCC).”— CMS MS-DRG Definitions Manual (Appendix C) · FY2026
  • DRG 813 — COAGULATION DISORDERS[MS-DRG]: “COAGULATION DISORDERS (MDC 16)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026

MDC crossing

  • MDC 16 — Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders[MDC crossing]: “Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 1,614 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries (9)

  • Christmas disease[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Deficiency, deficient, anti-hemophilic, factor (A), B[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Deficiency, deficient, autoprothrombin, II[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Deficiency, deficient, Christmas factor[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Deficiency, deficient, factor, IX (congenital) (functional) (hereditary) (with functional defect)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Deficiency, deficient, plasma thromboplastin, component (PTC)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Deficiency, deficient, PTC (plasma thromboplastin component)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Disease, diseased, Christmas[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • and 1 more

Change history

  • FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
Coding guidelines Official source data
ICD-10-CM Official Guidelines for Coding and Reporting (FY2026), quoted by section Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 Release, file and checksum · Publisher’s page
Risk adjustment Official source data
2026 Mid-Year Final ICD-10 Mappings + Model Software (cms.gov/files/zip/2026-midyear-final-icd-10-mappings.zip, 2026-midyear-final-model-software.zip) — CMS-HCC V28, PY2026 mid-year final release Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder structured relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "D67 — Hereditary factor IX deficiency." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/d67-hereditary-factor-ix-deficiency

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Hereditary factor IX deficiency

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027 (effective October 1, 2026), and none are recorded for this code. Note changes are tracked from FY2027 only.