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D55.21 ICD-10-CM Code: Anemia due to pyruvate kinase deficiency

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Inpatient Payment Groups (MS-DRG)

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43.0, Appendix B.

  • MS-DRG 811 — RED BLOOD CELL DISORDERS WITH MCC (MDC 16)
  • MS-DRG 812 — RED BLOOD CELL DISORDERS WITHOUT MCC (MDC 16)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Official Registry Overview & Definition

Anemia due to pyruvate kinase deficiency is a billable ICD-10-CM diagnosis code (D55.21).

Official Tabular Instructional Notes

Sequencing, inclusion, and exclusion notes published for D55.21 in the official ICD-10-CM tabular list.

Inclusion Terms

Alternative terms the tabular list files under this code.

  • PK deficiency anemia
  • Pyruvate kinase deficiency anemia

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Indexed Clinical Terms (4)

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code.

  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), deficiency, PK
  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), deficiency, pyruvate kinase
  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), hemolytic, nonspherocytic, congenital or hereditary NEC, pyruvate kinase deficiency
  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), pyruvate kinase deficiency

Frequently Compared Codes

The official Excludes notes on D55.21 name these codes. Each comparison page covers when the two can — or must not — be reported together.

Change history

  • FY2022 — 2021-10-01
    Added to the code set
    Anemia due to pyruvate kinase deficiency
    FY2022 changes

Verify Before Coding

  • No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim.

Relationships & Classification

Official ICD-10-CM tabular notes on other codes that name D55.21 or its code family.

32 Excludes1 notes across 2 chapters: E70 (via D55.-), E70-E88 (via D55.-), E71 (via D55.-), E72 (via D55.-), E73 (via D55.-), E74 (via D55.-), E74.4 (via D55.-), E75 (via D55.-), E76 (via D55.-), E77 (via D55.-) and 22 more.

Potential MS-DRG Relationships (FY2026)

Not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity.

Named in the grouper logic of 2 MS-DRGs: DRG 811 (MDC 16), DRG 812 (MDC 16).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):BLD002 — Hemolytic anemia (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Contextual Map

Every relationship of D55.21 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Hierarchy

  • D50-D89 — Chapter 3: Diseases of the Blood and Blood-forming Organs and Certain Disorders Involving the Immune Mechanism (D50-D89) (D50-D89) [Hierarchy]

Referenced by Excludes1 notes (32)

  • E70 — Disorders of aromatic amino-acid metabolism [Excludes1](via D55.-): “hemolytic anemias attributable to enzyme disorders (D55.-)” · check together
  • E70-E88 — Metabolic disorders (E70-E88) [Excludes1](via D55.-): “hemolytic anemias attributable to enzyme disorders (D55.-)” · check together
  • E71 — Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism [Excludes1](via D55.-): “hemolytic anemias attributable to enzyme disorders (D55.-)” · check together
  • E72 — Other disorders of amino-acid metabolism [Excludes1](via D55.-): “hemolytic anemias attributable to enzyme disorders (D55.-)” · check together
  • E73 — Lactose intolerance [Excludes1](via D55.-): “hemolytic anemias attributable to enzyme disorders (D55.-)” · check together
  • E74 — Other disorders of carbohydrate metabolism [Excludes1](via D55.-): “hemolytic anemias attributable to enzyme disorders (D55.-)” · check together
  • E74.4 — Disorders of pyruvate metabolism and gluconeogenesis [Excludes1](via D55.-): “disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)” · check together
  • E75 — Disorders of sphingolipid metabolism and other lipid storage disorders [Excludes1](via D55.-): “hemolytic anemias attributable to enzyme disorders (D55.-)” · check together
  • and 24 more

Clinical classification (CCSR)

  • BLD002 — Hemolytic anemia [CCSR]

Potential MS-DRG

  • DRG 811 — RED BLOOD CELL DISORDERS WITH MCC [MS-DRG]: “RED BLOOD CELL DISORDERS WITH MCC (MDC 16)”
  • DRG 812 — RED BLOOD CELL DISORDERS WITHOUT MCC [MS-DRG]: “RED BLOOD CELL DISORDERS WITHOUT MCC (MDC 16)”

MDC crossing · procedures (1614)

  • MDC 16 — Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders [MDC crossing]: “Diseases and Disorders of Blood, Blood Forming Organs and Immunologic Disorders — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs.”
  • 02HV02Z — Insertion of Monitoring Device into Superior Vena Cava, Open Approach [Same-MDC procedure]: “Insertion of Monitoring Device into Superior Vena Cava, Open Approach — grouped in MDC 16, the procedure side of this code's crossing.” · check together
  • 02HV0DZ — Insertion of Intraluminal Device into Superior Vena Cava, Open Approach [Same-MDC procedure]: “Insertion of Intraluminal Device into Superior Vena Cava, Open Approach — grouped in MDC 16, the procedure side of this code's crossing.” · check together
  • 02HV3DZ — Insertion of Intraluminal Device into Superior Vena Cava, Percutaneous Approach [Same-MDC procedure]: “Insertion of Intraluminal Device into Superior Vena Cava, Percutaneous Approach — grouped in MDC 16, the procedure side of this code's crossing.” · check together
  • 02HV42Z — Insertion of Monitoring Device into Superior Vena Cava, Percutaneous Endoscopic Approach [Same-MDC procedure]: “Insertion of Monitoring Device into Superior Vena Cava, Percutaneous Endoscopic Approach — grouped in MDC 16, the procedure side of this code's crossing.” · check together
  • 02HV4DZ — Insertion of Intraluminal Device into Superior Vena Cava, Percutaneous Endoscopic Approach [Same-MDC procedure]: “Insertion of Intraluminal Device into Superior Vena Cava, Percutaneous Endoscopic Approach — grouped in MDC 16, the procedure side of this code's crossing.” · check together
  • and 1609 more

Index entries

  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), deficiency, PK[Index term]
  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), deficiency, pyruvate kinase[Index term]
  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), hemolytic, nonspherocytic, congenital or hereditary NEC, pyruvate kinase deficiency[Index term]
  • Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound), pyruvate kinase deficiency[Index term]

Nearest codes

  • D55 — Anemia due to enzyme disorders [Sibling]
  • D55.0 — Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency [Sibling]
  • D55.1 — Anemia due to other disorders of glutathione metabolism [Sibling]
  • D55.2 — Anemia due to disorders of glycolytic enzymes [Sibling]
  • D55.29 — Anemia due to other disorders of glycolytic enzymes [Sibling]
  • D55.3 — Anemia due to disorders of nucleotide metabolism [Sibling]
  • D55.8 — Other anemias due to enzyme disorders [Sibling]
  • D55.9 — Anemia due to enzyme disorder, unspecified [Sibling]

Change history

  • FY2022 — Added to the code set [Change history]

Nearest Codes in This Family

Official ICD-10-CM classifications closest to D55.21 in its code family, with their registry titles.

  • D55 — Anemia due to enzyme disorders
  • D55.0 — Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
  • D55.1 — Anemia due to other disorders of glutathione metabolism
  • D55.2 — Anemia due to disorders of glycolytic enzymes
  • D55.29 — Anemia due to other disorders of glycolytic enzymes
  • D55.3 — Anemia due to disorders of nucleotide metabolism
  • D55.8 — Other anemias due to enzyme disorders
  • D55.9 — Anemia due to enzyme disorder, unspecified

View all codes in the D55 family