Anaplstc lg cell lymph, ALK-neg, extrnod and solid org sites

This code identifies a rare and aggressive type of non-Hodgkin lymphoma characterized by large, atypical lymphocytes that do not express the anaplastic lymphoma kinase (ALK) protein. The disease primarily affects sites outside of the lymph nodes, such as the skin, bone, soft tissue, or viscera.

Apply this code when documentation confirms a diagnosis of anaplastic large cell lymphoma that has been specifically tested and found to be ALK-negative, and the primary manifestation is in extranodal or solid organ sites. Pathological reports detailing ALK status and the location of the tumor are essential for accurate coding.

• Ensure the pathology report explicitly states "ALK-negative" to differentiate from ALK-positive variants.
• Verify the primary site of involvement is extranodal or a solid organ; do not use for nodal involvement.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter or treatment.