Short answer · Medically reviewed summary · Last updated: 2026-05-08
The primary ICD-10-CM code for Castleman disease is D47.Z2 (Castleman disease), while the historical ICD-9-CM code used for billing and classification was 289.89. These codes are essential for healthcare providers to document the specific clinical presentation of this rare lymphoproliferative disorder. What exactly is Castleman disease? Castleman disease is a rare group of lymphoproliferative disorders characterized by the enlargement of lymph nodes and immune system dysregulation.
ICD10 code of Castleman disease and ICD9 code
ICD-10 and ICD-9 codes for Castleman disease, with classification details for clinicians, coders and patients.
The primary ICD-10-CM code for Castleman disease is D47.Z2 (Castleman disease), while the historical ICD-9-CM code used for billing and classification was 289.89. These codes are essential for healthcare providers to document the specific clinical presentation of this rare lymphoproliferative disorder.
What exactly is Castleman disease?
Castleman disease is a rare group of lymphoproliferative disorders characterized by the enlargement of lymph nodes and immune system dysregulation. It is classified into two main clinical types: unicentric (affecting a single lymph node region) and multicentric (affecting multiple regions, often accompanied by systemic inflammatory symptoms). Because Castleman disease is complex, accurate coding is vital for coordinating multidisciplinary care.
How is Castleman disease classified for billing?
Medical coding for Castleman disease ensures that patients receive appropriate insurance coverage for diagnostic procedures and treatments. The following codes are standard in clinical settings:
- ICD-10-CM: D47.Z2 (Castleman disease)
- ICD-9-CM (Historical): 289.89 (Other specified diseases of blood and blood-forming organs)
Is Castleman disease hereditary?
Current research indicates that Castleman disease is generally not considered an inherited, genetic condition. While the exact cause remains under investigation, the idiopathic form of multicentric Castleman disease is often linked to cytokine storms, particularly the overproduction of Interleukin-6 (IL-6). Understanding the distinction between the localized and systemic forms of Castleman disease is crucial for determining the right treatment pathway.
Support for those living with the condition
Navigating a rare diagnosis can feel isolating. At DiseaseMaps.org, we have a community of 59 people with Castleman disease who share their experiences, symptom management strategies, and emotional support. Connecting with others who understand the clinical nuances of Castleman disease can be an essential part of your journey.
Next steps
- Consult with a hematologist or oncologist who specializes in Castleman disease.
- Verify that your healthcare provider uses the correct D47.Z2 code for your medical records.
- Join the Castleman disease community on DiseaseMaps.org to connect with others.
- Review the latest clinical trial information through the Castleman Disease Collaborative Network (CDCN).
Medical disclaimer: This content is for informational purposes only and does not constitute professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Castleman disease
- Orphanet: Rare disease database, ORPHA:135
- Castleman Disease Collaborative Network (CDCN)
- ICD-10 Data: D47.Z2
