D47.Z2

HCC Category Mapping

What This Code Means

An abnormal growth of lymphoid tissue characterized by enlarged lymph nodes and excessive antibody production, with uncertain malignant potential.

Coding Tips

• •Ensure pathology confirms Castleman disease diagnosis; distinguish between unicentric and multicentric variants in documentation
• •Document any associated symptoms like fever, anemia, or elevated inflammatory markers

Clinical Significance

Castleman disease (also known as angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder characterized by abnormal lymph node enlargement with characteristic histopathology. Unicentric disease involves a single lymph node region and is often curable with surgical resection, while multicentric Castleman disease is a systemic inflammatory condition with significant morbidity driven by interleukin-6 dysregulation.

Documentation Requirements

• ✓Documentation must include tissue biopsy confirming Castleman disease histology (hyaline vascular, plasma cell, or mixed type).
• ✓Specify unicentric versus multicentric disease.
• ✓For multicentric disease, document systemic symptoms (fever, night sweats, weight loss), laboratory findings (elevated interleukin-6, C-reactive protein, anemia), and human herpesvirus 8 status.
• ✓Treatment regimen (siltuximab, rituximab) should be documented.

Commonly Confused Codes