ICD-10 Code D64.4 – Congenital dyserythropoietic anemia (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Congenital dyserythropoietic anemia is D64.4.
2026 ICD-10-CM Diagnosis Code D64.4 – Congenital dyserythropoietic anemia

What it is

Congenital dyserythropoietic anemia is an inherited disorder of red blood cell production. It causes ineffective erythropoiesis, so the body makes abnormal red cells and anemia develops.

Clinical signs

Clinical features vary; refer to documentation. Common findings include chronic anemia, jaundice, and splenomegaly, with laboratory evidence of ineffective red cell production.

When to use this code

Use D64.4 when the record documents congenital dyserythropoietic anemia by name or confirms a hereditary dyserythropoietic anemia. Code it for encounters focused on evaluation, monitoring, or management of this specific diagnosis.

Do not use it for nonspecific anemia or for acquired causes of red cell production problems. If the chart only says “anemia” or “hemolytic anemia,” check documentation for a more precise diagnosis.

Do not use for

Do not assign this code for iron deficiency anemia, aplastic anemia, or other acquired anemias. Check documentation if the condition is not explicitly congenital or dyserythropoietic.

Coding tip

Confirm that the provider documents the congenital dyserythropoietic type, not just anemia, before selecting D64.4.

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