Anaplastic large cell lymphoma, ALK-negative, spleen

This code signifies 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 specific anatomical site of involvement for this diagnosis is the spleen.

Use this code when documentation clearly states a diagnosis of anaplastic large cell lymphoma that has been confirmed as ALK-negative, with primary or significant involvement of the spleen. This diagnosis is typically established through biopsy and immunohistochemical staining.

• Ensure the pathology report explicitly states "ALK-negative" to differentiate from ALK-positive variants.
• Verify splenic involvement is documented; if other sites are involved, additional codes may be necessary.
• Do not confuse with other types of non-Hodgkin lymphoma or other anaplastic large cell lymphomas without the ALK-negative specification.