Sezary disease, lymph nodes of multiple sites

This code represents Sezary disease, a rare and aggressive form of cutaneous T-cell lymphoma, specifically when it has metastasized to or primarily involves multiple lymph node regions. It is characterized by erythroderma, generalized lymphadenopathy, and the presence of Sezary cells in the skin, lymph nodes, and peripheral blood.

Use this code when documentation confirms a diagnosis of Sezary disease with involvement of multiple lymph node sites. This typically requires biopsy confirmation of Sezary cells in more than one lymph node region or clear clinical evidence of widespread lymphadenopathy attributed to Sezary disease.

• Ensure documentation specifies "multiple sites" for lymph node involvement; if only a single site is documented, a more specific code should be used.
• Distinguish from other cutaneous T-cell lymphomas, such as Mycosis Fungoides, even if lymph node involvement is present.
• Sequence this code as the primary diagnosis when Sezary disease is the reason for the encounter and lymph node involvement is a significant aspect.