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D61.02

Billable

Shwachman-Diamond syndrome

Last updated: FY2026 ICD-10-CM (Oct 1, 2025 – Sep 30, 2026) | CMS-HCC V28 (100% phase-in, PY2026)

Is D61.02 an HCC code?

Yes. D61.02 maps to Hemolytic and Aplastic Anemias under the CMS-HCC V28 risk adjustment model (and Severe Hematological Disorders under V24).

HCC Category Mapping

V28HCC 109Hemolytic and Aplastic Anemias
0.291
V24HCC 46Severe Hematological Disorders
0.666
ESRDHCC 46Severe Hematological Disorders
0.000
RxHCCHCC 96Hemolytic 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 D61.02

For D61.02 to 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 D61.02 during that encounter — not just copy-forwarded from a problem list.

What This Code Means

D61.02 is the ICD-10-CM diagnosis code for shwachman-diamond syndrome. A rare inherited disorder characterized by bone marrow failure, short stature, and pancreatic insufficiency affecting fat digestion. D61.02 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, D61.02 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, D61.02 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.

This is a specific syndrome; document all associated features (pancreatic dysfunction, skeletal abnormalities, growth retardation) when present. Because D61.02 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 D61.02 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • This is a specific syndrome; document all associated features (pancreatic dysfunction, skeletal abnormalities, growth retardation) when present
  • Genetic testing confirmation should be noted in the medical record to support this diagnosis

Clinical Significance

Shwachman-Diamond syndrome is a rare autosomal recessive inherited bone marrow failure disorder caused primarily by mutations in the SBDS gene, characterized by the triad of exocrine pancreatic insufficiency, bone marrow failure (typically neutropenia as the predominant cytopenia), and skeletal abnormalities. It is the second most common cause of inherited exocrine pancreatic insufficiency after cystic fibrosis. Patients face significant risks of progression to myelodysplastic syndrome and acute myeloid leukemia, with transformation rates estimated at 15-25% over the lifetime.

Documentation Requirements

  • Document genetic testing confirming SBDS mutation or clinical diagnosis based on characteristic features.
  • Record evidence of pancreatic insufficiency (low fecal elastase, steatorrhea, fat-soluble vitamin deficiencies).
  • Include complete blood count showing cytopenias (especially neutropenia), bone marrow biopsy findings, and skeletal imaging showing metaphyseal chondrodysplasia.
  • Document growth parameters and nutritional status.
  • Note any evidence of myelodysplastic transformation on bone marrow surveillance.

Use Additional Code

  • code, if applicable, for genetic susceptibility to other malignant neoplasm (Z15.09)

Code Also

  • , if applicable, associated conditions such as:
  • acute myeloblastic leukemia (C92.0-)
  • exocrine pancreatic insufficiency (K86.81)
  • myelodysplastic syndrome (D46.-)

Commonly Confused Codes

Code Hierarchy

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