ICD-10 Code D47.Z2 – Castleman disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D47.Z2 – Castleman disease
What it is
D47.Z2 identifies Castleman disease, a rare lymph node disorder that may be unicentric or multicentric. Use it when the record documents Castleman disease specifically, rather than another lymphoproliferative or inflammatory condition.
Clinical signs
Clinical features vary; refer to documentation. Common findings may include enlarged lymph nodes, constitutional symptoms, and laboratory or organ findings consistent with the documented Castleman disease subtype.
When to use this code
Use D47.Z2 when the provider diagnoses Castleman disease and the note supports that diagnosis. It applies to both localized and generalized forms if the documentation does not specify a more detailed coded subtype. If the record only suggests lymphadenopathy without confirmation, check documentation before coding.
Do not use for
Do not use this code for nonspecific lymph node enlargement, suspected disease, or other confirmed lymphoproliferative disorders. If the chart names a different condition, code that diagnosis instead.
Coding tip
Look for the exact phrase “Castleman disease” in the assessment or problem list, and code only what the provider has clearly documented.