Sezary disease, extranodal and solid organ sites

This code signifies Sezary disease, a rare and aggressive form of cutaneous T-cell lymphoma, when it has spread beyond the skin to involve extranodal sites (e.g., lymph nodes, bone marrow) and/or solid organs. It indicates advanced systemic involvement of this malignancy.

Use this code when documentation confirms a diagnosis of Sezary disease with evidence of metastasis to internal organs or non-lymphatic tissues outside the skin. This typically follows initial diagnosis and staging that reveals systemic dissemination. Supporting documentation includes pathology reports, imaging studies, and physician notes detailing extranodal or solid organ involvement.

• Ensure physician documentation clearly specifies "extranodal" or "solid organ" involvement for Sezary disease, not just cutaneous or lymph node involvement.
• Do not use this code for Sezary disease limited to the skin or lymph nodes; other codes exist for those presentations.
• Sequence this code as the primary diagnosis when it is the reason for the encounter, followed by codes for specific extranodal or solid organ manifestations if applicable.