D55.1 ICD-10-CM Code: Anemia due to other disorders of glutathione metabolism
D55.1 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · free HCC coding tools
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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) / Hemolytic anemias (D55-D59)
D55.1
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceAnemia due to other disorders of glutathione metabolism
A rare type of anemia resulting from problems with glutathione metabolism, which is important for protecting red blood cells from oxidative damage. This leads to reduced red blood cell survival and anemia.
Buddy Insight
This rare hemolytic anemia results from defects in glutathione metabolism pathways other than Glucose-6-phosphate dehydrogenase deficiency.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
N/A—
Not mapped
ESRD/PACE
MappedHCC 48
Code-level coefficient reference
RXHCC
N/A—
Not mapped
Code Book Path
Inclusion Terms
Official- Anemia (due to) enzyme deficiencies, except G6PD, related to the hexose monophosphate [HMP] shunt pathway
- Anemia (due to) hemolytic nonspherocytic (hereditary), type I
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
Related Codes
Includes
OfficialNo Includes notes are included in this display for D55.1. 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
OfficialNo Code First sequencing instructions are included in this display for D55.1. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for D55.1. Check the code and parent instructions in the Code Book.
Code Also
OfficialNo Code Also instructions are included in this display for D55.1. Check the code and parent instructions in the Code Book.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
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 D55.1 an HCC code?
D55.1 has no mapping under the current CMS-HCC V28 community payment model. D55.1 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
- D55.1
- Description
- Anemia due to other disorders of glutathione metabolism
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF reference coefficient
- —
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
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 D55.1 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for D55.1
For D55.1, 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
D55.1 is the ICD-10-CM diagnosis code for anemia due to other disorders of glutathione metabolism. A rare type of anemia resulting from problems with glutathione metabolism, which is important for protecting red blood cells from oxidative damage. This leads to reduced red blood cell survival and anemia. D55.1 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).
D55.1 has no mapping under the current CMS-HCC V28 community payment model. D55.1 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 a rare condition; ensure documentation clearly supports the specific glutathione metabolism disorder.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for D55.1 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is a rare condition; ensure documentation clearly supports the specific glutathione metabolism disorder
- •Differentiate from G6PD deficiency (D55.0) and other enzyme disorders
Clinical Significance
This rare hemolytic anemia results from defects in glutathione metabolism pathways other than Glucose-6-phosphate dehydrogenase deficiency. Glutathione is critical for protecting red blood cells from oxidative damage, and deficiency leads to chronic or episodic hemolysis. These conditions are typically inherited in an autosomal recessive pattern and may present with neonatal jaundice, chronic hemolytic anemia, or intermittent crisis episodes.
Documentation Requirements
- ✓Documentation must specify the glutathione metabolism disorder identified (e.g., glutathione synthetase deficiency, gamma-glutamylcysteine synthetase deficiency), supported by enzyme assays or genetic testing.
- ✓Record hemoglobin levels, reticulocyte counts, evidence of hemolysis (elevated lactate dehydrogenase, indirect bilirubin, haptoglobin), and the management plan including folic acid supplementation and transfusion history.
Commonly Confused Codes
- •D55.1 vs. D55.0 (Glucose-6-phosphate dehydrogenase deficiency) -
- •Glucose-6-phosphate dehydrogenase deficiency has its own specific code and should not be coded here. D55.1 vs. D55.8 (Other anemias due to enzyme disorders) -
- •use D55.1 only when the disorder is specifically in the glutathione metabolism pathway. D55.1 vs. D58.8 (Other specified hereditary hemolytic anemias) -
- •D58.8 covers membrane and other non-enzyme hereditary causes.