ICD-10 Code D55.21 – Anemia due to pyruvate kinase deficiency (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Anemia due to pyruvate kinase deficiency is D55.21.
2026 ICD-10-CM Diagnosis Code D55.21 – Anemia due to pyruvate kinase deficiency

What it is

This code identifies anemia caused by inherited pyruvate kinase deficiency, a red blood cell enzyme disorder that leads to premature cell breakdown. Use it when documentation links the anemia directly to pyruvate kinase deficiency.

Clinical signs

Clinical features vary; refer to documentation. Commonly noted findings include chronic hemolytic anemia, jaundice, splenomegaly, and fatigue, but you should code only when the diagnosis is clearly documented.

When to use this code

Use D55.21 when the provider documents anemia due to pyruvate kinase deficiency, including hereditary hemolytic anemia attributed to this enzyme defect. It also fits follow-up encounters when the condition remains active and is being managed or monitored. Check documentation if the record mentions pyruvate kinase deficiency without stating anemia.

Do not use for

Do not use this code for other hemolytic anemias, nonspecific anemia, or anemia from a different cause. If the chart does not clearly connect the anemia to pyruvate kinase deficiency, check documentation.

Coding tip

Code the specific cause only when the provider explicitly links anemia to pyruvate kinase deficiency.

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