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ICD-10-CM 2027

ICD-10 code for pyruvate kinase deficiency anemia

D55.21

Anemia due to pyruvate kinase deficiency

✓ Billable / specific

From the official ICD-10-CM alphabetic index entry “Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound) › pyruvate kinase deficiency”. Page updated September 29, 2026.

About coding pyruvate kinase deficiency anemia

The ICD-10-CM code for pyruvate kinase deficiency anemia is D55.21 (Anemia due to pyruvate kinase deficiency).

Within D55.2 (anemia due to disorders of glycolytic enzymes), D55.21 is specifically for anemia due to pyruvate kinase deficiency. Related codes cover other disorders of glycolytic enzymes (D55.29).

It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.

In MS-DRG v44.0, it is part of the grouping logic for DRG 811 (Red Blood Cell Disorders with MCC, relative weight 1.3988) and DRG 812 (Red Blood Cell Disorders without MCC, relative weight 0.9234), in MDC 16 (Diseases and Disorders of the Blood and Blood Forming Organs and Immunological Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

Common questions about pyruvate kinase deficiency anemia ICD-10 codes

What is the ICD-10 code for pyruvate kinase deficiency anemia?

D55.21 — Anemia due to pyruvate kinase deficiency.

Is D55.21 billable?

Yes. D55.21 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can D55.21 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict D55.21 as a principal diagnosis.

Is D55.21 a CC or MCC?

No. D55.21 is neither a CC nor an MCC under MS-DRG v44.0.