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D55.9 ICD-10-CM Code: Anemia due to enzyme disorder, unspecified

D55.9 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · HCC coding software

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Code lookupD55.9

FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Hemolytic anemias (D55-D59)

D55.9

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

Anemia due to enzyme disorder, unspecified

A type of anemia caused by an enzyme disorder, but the specific enzyme deficiency has not been identified or documented. This is a placeholder code when the exact cause is unknown.

Buddy the Bee presenting code insight

Buddy Insight

This unspecified code is used when hemolytic anemia is attributed to an enzyme disorder but the specific enzyme deficiency has not been identified or documented.

CMS-HCC V28

N/A

Not mapped

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

N/A

Not mapped

ESRD/PACE

HCC 48

Code-level coefficient reference

RXHCC

N/A

Not mapped

Inclusion Terms

Official

No inclusion terms are included in this display for D55.9. Check the code and parent instructions in the Code Book.

Excludes 2

Official
  • autoimmune disease (systemic) NOS (M35.9)Inherited from D50-D89
  • certain conditions originating in the perinatal period (P00-P96)Inherited from D50-D89
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)Inherited from D50-D89
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)Inherited from D50-D89
  • endocrine, nutritional and metabolic diseases (E00-E88)Inherited from D50-D89
  • human immunodeficiency virus [HIV] disease (B20)Inherited from D50-D89
  • injury, poisoning and certain other consequences of external causes (S00-T88)Inherited from D50-D89
  • neoplasms (C00-D49)Inherited from D50-D89
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)Inherited from D50-D89

Includes

Official

No Includes notes are included in this display for D55.9. Check the code and parent instructions in the Code Book.

Excludes 1

Official
  • drug-induced enzyme deficiency anemia (D59.2)Inherited from D55

Code First

Official

No Code First sequencing instructions are included in this display for D55.9. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for D55.9. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for D55.9. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
Documentation should include the basis for suspecting an enzyme disorder (e.g., chronic hemolysis with negative Coombs test, family history), results of any enzyme assays performed, and the reason why a more specific enzyme cannot be identified.
Record hemoglobin levels, reticulocyte counts, and management plan.
A provider query should be initiated to obtain additional specificity whenever possible.

MEAT Support

HCC Buddy guidance
Documentation should include the basis for suspecting an enzyme disorder (e.g., chronic hemolysis with negative Coombs test, family history), results of any enzyme assays performed, and the reason why a more specific enzyme cannot be identified.
Record hemoglobin levels, reticulocyte counts, and management plan.
A provider query should be initiated to obtain additional specificity whenever possible.

Audit Caution

HCC Buddy guidance
This is the least specific code in the D55 series and should only be used when more specific information cannot be obtained. No V28 HCC mapping exists for this code. Always query the provider for the specific enzyme disorder before assigning D55.
Avoid using this code long-term -- repeated encounters should prompt additional diagnostic workup to achieve more specific classification.

Common Mistakes

HCC Buddy guidance
D55.9 vs. D55.8 (Other anemias due to enzyme disorders) -
use D55.8 when the enzyme disorder is identified but does not fit a specific subcategory, vs. D55.9 when completely unknown. D55.9 vs. D58.9 (Hereditary hemolytic anemia, unspecified) -
D58.9 covers all hereditary hemolytic anemias unspecified, not limited to enzyme defects. D55.9 vs. D59.9 (Acquired hemolytic anemia, unspecified) -
ensure the disorder is documented as inherited, not acquired.

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is D55.9 an HCC code?

D55.9 is not in the CMS-HCC V28 or V24 community payment model. D55.9 has a separate mapping under the CMS-HCC ESRD model (HCC 48 (Coagulation Defects and Other Specified Hematological Disorders)); the applicable result needs member context.

Code
D55.9
Description
Anemia due to enzyme disorder, unspecified
HCC (V28)
No CMS-HCC V28 mapping
RAF reference coefficient
Billable
Yes
Payment year
2026

HCC Category Mapping

ESRDHCC 48, Coagulation Defects and Other Specified Hematological Disorders
Not separately weighted

These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.

Work D55.9 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for D55.9

For D55.9, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

  • 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

Coder workflow notes

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What This Code Means

D55.9 is the ICD-10-CM diagnosis code for anemia due to enzyme disorder, unspecified. A type of anemia caused by an enzyme disorder, but the specific enzyme deficiency has not been identified or documented. This is a placeholder code when the exact cause is unknown. D55.9 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 hemolytic anemias (d55-d59).

D55.9 has no mapping under the CMS-HCC V28 or V24 community payment models. D55.9 has a separate mapping under the CMS-HCC ESRD model (HCC 48 (Coagulation Defects and Other Specified Hematological Disorders)); the applicable result needs member context. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.

This is the least specific code in the D55 category; use only when the specific enzyme disorder cannot be determined.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for D55.9 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • This is the least specific code in the D55 category; use only when the specific enzyme disorder cannot be determined
  • Query the provider to obtain more specific diagnosis if possible to allow for more precise coding

Clinical Significance

This unspecified code is used when hemolytic anemia is attributed to an enzyme disorder but the specific enzyme deficiency has not been identified or documented. It represents the least specific code in the D55 category and should prompt a query to the provider for additional diagnostic workup. The clinical impact of unspecified enzyme disorders varies widely, making accurate classification important for appropriate management and risk stratification.

Documentation Requirements

  • Documentation should include the basis for suspecting an enzyme disorder (e.g., chronic hemolysis with negative Coombs test, family history), results of any enzyme assays performed, and the reason why a more specific enzyme cannot be identified.
  • Record hemoglobin levels, reticulocyte counts, and management plan.
  • A provider query should be initiated to obtain additional specificity whenever possible.

Commonly Confused Codes

  • D55.9 vs. D55.8 (Other anemias due to enzyme disorders) -
  • use D55.8 when the enzyme disorder is identified but does not fit a specific subcategory, vs. D55.9 when completely unknown. D55.9 vs. D58.9 (Hereditary hemolytic anemia, unspecified) -
  • D58.9 covers all hereditary hemolytic anemias unspecified, not limited to enzyme defects. D55.9 vs. D59.9 (Acquired hemolytic anemia, unspecified) -
  • ensure the disorder is documented as inherited, not acquired.

Child Codes

Code Hierarchy

Work D55.9 in HCC Buddy

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