Castleman disease

This code represents Castleman disease, a rare lymphoproliferative disorder characterized by the overgrowth of lymphatic tissue. It can manifest as a single enlarged lymph node (unicentric) or affect multiple lymph nodes and organs throughout the body (multicentric). The disease is often associated with systemic symptoms like fever, weight loss, and fatigue.

Use this code when the physician documentation explicitly states a diagnosis of Castleman disease, without further specification of its type (e.g., unicentric, multicentric, hyaline-vascular, plasma cell). This code is appropriate for both initial diagnoses and follow-up care for patients with this condition. Documentation should clearly indicate the presence of Castleman disease.

• Do not use this code if the documentation specifies a particular type of Castleman disease; instead, look for more specific codes within the D47 category.
• Be mindful of the Excludes2 note for Kaposi's sarcoma (C46.-), as these are distinct conditions.
• Code any associated symptoms or complications, such as anemia or organomegaly, in addition to the Castleman disease.