Heavy chain disease not having achieved remission

This code signifies a rare lymphoproliferative disorder characterized by the production of abnormal, truncated immunoglobulin heavy chains without associated light chains. It represents a persistent state of the disease where the patient has not achieved a complete or partial remission following treatment.

Apply this code when documentation clearly indicates a diagnosis of heavy chain disease (e.g., Alpha, Gamma, or Mu heavy chain disease) and explicitly states that the patient is not in remission. This is typically used for ongoing management of the disease or when treatment has failed to induce remission.

• Ensure the medical record specifies "not in remission" or similar language indicating active, persistent disease.
• Do not use this code if the patient has achieved remission, even if they are still receiving maintenance therapy.
• Consider sequencing this code as a primary diagnosis if it is the main reason for the encounter, or as a secondary diagnosis if managing complications of the disease.