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D61.03 ICD-10-CM Code: Fanconi anemia

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FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Aplastic and other anemias and other bone marrow failure syndromes (D60-D64)

D61.03

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Fanconi anemia

Fanconi anemia is a rare inherited blood disorder that affects the bone marrow's ability to produce blood cells, leading to low counts of red blood cells, white blood cells, and platelets. Patients with this condition have an increased risk of developing certain cancers and may experience fatigue, infections, and bleeding problems.

Buddy the Bee presenting code insight

Buddy Insight

Fanconi anemia is a rare autosomal recessive (or rarely X-linked) inherited bone marrow failure syndrome caused by mutations in DNA repair pathway genes, characterized by progressive pancytopenia, congenital anomalies, and a markedly elevated risk of malignancies.

CMS-HCC V28

HCC 109

RAF 0.291

CMS-HCC V24

HCC 46

RAF 0.666

ACA/HHS

0

0

RAF 0

ESRD/PACE

HCC 46

RAF 0.0

RXHCC

HCC 96

RAF 0.0

Code Trumping

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Code Book Path

Official
D61Other aplastic anemias and other bone marrow failure syndromes
D61.0Constitutional aplastic anemia
D61.03Fanconi anemia

Inclusion Terms

Official
  • Fanconi pancytopenia
  • Fanconi's anemia

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for D61.03 in this effective period.

Related Child Codes

Official
D61.01Constitutional (pure) red blood cell aplasia
D61.02Shwachman-Diamond syndrome
D61.09Other constitutional aplastic anemia

Includes

Official

ICD-10-CM does not list Includes notes for D61.03 in this effective period.

Excludes 1

Official
  • Fanconi syndrome (E72.0-)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for D61.03 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for D61.03 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for D61.03 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Document chromosomal breakage analysis (mitomycin C or diepoxybutane testing) confirming Fanconi anemia diagnosis.
Record the specific complementation group (FANCA through FANCQ) when genetic testing has been performed.
Include complete blood count trends showing progressive cytopenias, bone marrow biopsy findings, and any congenital anomalies documented on physical examination or imaging.
Document treatment including androgen therapy, hematopoietic growth factors, transfusion history, and stem cell transplant status.

MEAT Support

HCC Buddy guidance
Document chromosomal breakage analysis (mitomycin C or diepoxybutane testing) confirming Fanconi anemia diagnosis.
Record the specific complementation group (FANCA through FANCQ) when genetic testing has been performed.
Include complete blood count trends showing progressive cytopenias, bone marrow biopsy findings, and any congenital anomalies documented on physical examination or imaging.
Document treatment including androgen therapy, hematopoietic growth factors, transfusion history, and stem cell transplant status.

Audit Caution

HCC Buddy guidance
Do not confuse Fanconi anemia (a bone marrow failure syndrome) with Fanconi syndrome (a renal tubular disorder, E72.09) -
these are completely different conditions. Code any associated congenital anomalies and malignancies separately. The progressive nature means annual documentation should reflect current disease severity and complications. Chromosomal breakage testing is the gold standard diagnostic test -
clinical features alone may not be sufficient for this specific code. Patients who have received stem cell transplant still carry the genetic condition and remain at risk for solid tumors.

Common Mistakes

HCC Buddy guidance
D61.01 (Constitutional pure red blood cell aplasia) affects only red cells, not all lineages.
D61.02 (Shwachman-Diamond syndrome) involves pancreatic insufficiency without DNA repair defects.
D61.09 (Other constitutional aplastic anemia) is less specific and should not be used when Fanconi anemia is confirmed.
D46.x (Myelodysplastic syndromes) may develop as a complication but is a separate diagnosis requiring additional coding.

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is D61.03 an HCC code?

Yes. D61.03 maps to Hemolytic and Aplastic Anemias under the CMS-HCC V28 risk adjustment model (and Severe Hematological Disorders under V24).

HCC Category Mapping

V28HCC 109, Hemolytic and Aplastic Anemias
0.291
V24HCC 46, Severe Hematological Disorders
0.666
ESRDHCC 46, Severe Hematological Disorders
0.000
RxHCCHCC 96, Hemolytic and Aplastic Anemias
0.000

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

MEAT Criteria for D61.03

For D61.03to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed D61.03 during that encounter, not just copy-forwarded from a problem list.

What This Code Means

D61.03 is the ICD-10-CM diagnosis code for fanconi anemia. Fanconi anemia is a rare inherited blood disorder that affects the bone marrow's ability to produce blood cells, leading to low counts of red blood cells, white blood cells, and platelets. Patients with this condition have an increased risk of developing certain cancers and may experience fatigue, infections, and bleeding problems. D61.03 sits in the ICD-10-CM chapter for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (d50-d89), within the section covering aplastic and other anemias and other bone marrow failure syndromes (d60-d64).

Under the CMS-HCC V28 risk adjustment model, D61.03 maps to Hemolytic and Aplastic Anemias (HCC 109) with a community, non-dual, aged base RAF weight of 0.291. Under the older CMS-HCC V24 model, D61.03 maps to Severe Hematological Disorders (HCC 46) with a community, non-dual, aged base RAF weight of 0.666. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

Fanconi anemia is a genetic disorder; verify documentation specifies the type or subtype if available, as there are multiple complementation groups (A through Q). Because D61.03 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for D61.03 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Fanconi anemia is a genetic disorder; verify documentation specifies the type or subtype if available, as there are multiple complementation groups (A through Q)
  • When coding Fanconi anemia, look for associated complications such as cytopenias, infections, or malignancies that should be coded separately to capture the full clinical picture

Clinical Significance

Fanconi anemia is a rare autosomal recessive (or rarely X-linked) inherited bone marrow failure syndrome caused by mutations in DNA repair pathway genes, characterized by progressive pancytopenia, congenital anomalies, and a markedly elevated risk of malignancies. Physical findings may include short stature, cafe-au-lait spots, thumb and radial ray abnormalities, renal malformations, and microcephaly, though up to 30% of patients have no visible anomalies. The median age of bone marrow failure onset is 7 years, with lifetime risks of approximately 50% for acute myeloid leukemia and 25% for head and neck squamous cell carcinomas.

Documentation Requirements

  • Document chromosomal breakage analysis (mitomycin C or diepoxybutane testing) confirming Fanconi anemia diagnosis.
  • Record the specific complementation group (FANCA through FANCQ) when genetic testing has been performed.
  • Include complete blood count trends showing progressive cytopenias, bone marrow biopsy findings, and any congenital anomalies documented on physical examination or imaging.
  • Document treatment including androgen therapy, hematopoietic growth factors, transfusion history, and stem cell transplant status.
  • Note any malignancy surveillance performed.

Excludes 1, Do NOT code together

  • Fanconi syndrome (E72.0-)

Commonly Confused Codes

  • D61.01 (Constitutional pure red blood cell aplasia) affects only red cells, not all lineages.
  • D61.02 (Shwachman-Diamond syndrome) involves pancreatic insufficiency without DNA repair defects.
  • D61.09 (Other constitutional aplastic anemia) is less specific and should not be used when Fanconi anemia is confirmed.
  • D46.x (Myelodysplastic syndromes) may develop as a complication but is a separate diagnosis requiring additional coding.

Child Codes

Code Hierarchy

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