What is ICD-10 code D61.01?

D61.01 is the ICD-10-CM diagnosis code for Constitutional (pure) red blood cell aplasia. A rare inherited condition present from birth where the bone marrow fails to produce red blood cells due to genetic factors. D61.01 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. D61.01 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. D61.01 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. D61.01 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

What is the V28 vs V24 difference for D61.01?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. For D61.01, V28 maps to Hemolytic and Aplastic Anemias at RAF 0.291 while V24 mapped to Severe Hematological Disorders at RAF 0.666. Both models can still appear in legacy data and in payer-specific lookback periods.

What should coders know when reporting D61.01?

This code indicates a congenital/inherited condition; document family history and genetic testing results if available Distinguish from acquired forms by confirming the condition's presence from early childhood or infancy Because D61.01 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

D61.01 ICD-10 Code: Constitutional (pure) red

ICD-10-CM Code View

HCC Buddy Code Card

Digital ICD-10 code-book layout with official code detail, always-visible risk models, Code Trumping, and Buddy coding guidance.

Code lookupD61.01Effective periodFY2026 Apr Update (Apr 1-Sep 30)

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)

D61.01

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

Constitutional (pure) red blood cell aplasia

A rare inherited condition present from birth where the bone marrow fails to produce red blood cells due to genetic factors.

Buddy Insight

CMS-HCC V28

Mapped

HCC 109

Hemolytic and Aplastic Anemias

RAF 0.291

CMS-HCC V24

Mapped

HCC 46

Severe Hematological Disorders

RAF 0.666

ACA/HHS

Not risk-adjusted for this model/period.

RAF 0

ESRD/PACE

Mapped

HCC 46

Severe Hematological Disorders

RAF 0.0

RXHCC

Mapped

HCC 96

Hemolytic and Aplastic Anemias

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

D61.01Constitutional (pure) red blood cell aplasia

Inclusion Terms

Official

Blackfan-Diamond syndrome
Congenital (pure) red cell aplasia
Familial hypoplastic anemia
Primary (pure) red cell aplasia
Red cell (pure) aplasia of infants

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for D61.01 in this effective period.

Related Child Codes

Official

D61.02Shwachman-Diamond syndrome

D61.03Fanconi anemia

D61.09Other constitutional aplastic anemia

Includes

Official

ICD-10-CM does not list Includes notes for D61.01 in this effective period.

Excludes 1

Official

acquired red cell aplasia (D60.9)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for D61.01 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for D61.01 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for D61.01 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Document the congenital/inherited nature confirmed by early-childhood onset and genetic testing when available.

Record hemoglobin levels, reticulocyte count, elevated erythrocyte adenosine deaminase activity (a diagnostic marker), and bone marrow biopsy showing selective erythroid hypoplasia.

Include any associated congenital anomalies.

Document treatment approach (corticosteroid therapy, chronic transfusion program, or stem cell transplant considerations) and response.

MEAT Support

HCC Buddy guidance

Document the congenital/inherited nature confirmed by early-childhood onset and genetic testing when available.

Record hemoglobin levels, reticulocyte count, elevated erythrocyte adenosine deaminase activity (a diagnostic marker), and bone marrow biopsy showing selective erythroid hypoplasia.

Include any associated congenital anomalies.

Document treatment approach (corticosteroid therapy, chronic transfusion program, or stem cell transplant considerations) and response.

Audit Caution

HCC Buddy guidance

Do not confuse with acquired pure red cell aplasia in adults, which has different etiologies and treatment approaches. Ensure documentation confirms the constitutional nature through early onset, genetic testing, or characteristic clinical features. Code associated congenital anomalies separately when present. Patients who achieve corticosteroid remission still carry the genetic condition and remain at risk for malignancy -

the diagnosis should be maintained in the problem list.

Common Mistakes

HCC Buddy guidance

D60.0 (Chronic acquired pure red cell aplasia) is acquired, not constitutional.

D61.03 (Fanconi anemia) is a different inherited bone marrow failure syndrome affecting all cell lines.

D61.09 (Other constitutional aplastic anemia) is a less specific inherited aplasia code.

D61.02 (Shwachman-Diamond syndrome) involves neutropenia and pancreatic insufficiency rather than isolated red cell failure.

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 D61.01 an HCC code?

Yes. D61.01 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 D61.01

For D61.01to 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 D61.01 during that encounter, not just copy-forwarded from a problem list.

What This Code Means

D61.01 is the ICD-10-CM diagnosis code for constitutional (pure) red blood cell aplasia. A rare inherited condition present from birth where the bone marrow fails to produce red blood cells due to genetic factors. D61.01 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, D61.01 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, D61.01 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 code indicates a congenital/inherited condition; document family history and genetic testing results if available. Because D61.01 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 D61.01 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•This code indicates a congenital/inherited condition; document family history and genetic testing results if available
•Distinguish from acquired forms by confirming the condition's presence from early childhood or infancy

Clinical Significance

Constitutional (pure) red blood cell aplasia, also known as Diamond-Blackfan anemia, is a rare inherited bone marrow failure syndrome characterized by selective failure of red blood cell production presenting in infancy or early childhood. Approximately 25% of patients have associated congenital anomalies including craniofacial defects, upper limb abnormalities (particularly thumb anomalies), cardiac defects, and short stature. The condition is caused by mutations in ribosomal protein genes and carries an increased lifetime risk of myelodysplastic syndrome, acute myeloid leukemia, and solid tumors.

Documentation Requirements

✓Document the congenital/inherited nature confirmed by early-childhood onset and genetic testing when available.
✓Record hemoglobin levels, reticulocyte count, elevated erythrocyte adenosine deaminase activity (a diagnostic marker), and bone marrow biopsy showing selective erythroid hypoplasia.
✓Include any associated congenital anomalies.
✓Document treatment approach (corticosteroid therapy, chronic transfusion program, or stem cell transplant considerations) and response.
✓Note any family history of the condition.

Excludes 1, Do NOT code together

acquired red cell aplasia (D60.9)

Commonly Confused Codes

•D60.0 (Chronic acquired pure red cell aplasia) is acquired, not constitutional.
•D61.03 (Fanconi anemia) is a different inherited bone marrow failure syndrome affecting all cell lines.
•D61.09 (Other constitutional aplastic anemia) is a less specific inherited aplasia code.
•D61.02 (Shwachman-Diamond syndrome) involves neutropenia and pancreatic insufficiency rather than isolated red cell failure.