Immunoproliferative small intestinal disease not remission

This code signifies a malignant lymphoproliferative disorder primarily affecting the small intestine, characterized by the proliferation of abnormal plasma cells or lymphocytes. It is a form of extranodal lymphoma, often associated with chronic inflammation or infection, and has not yet responded to treatment to achieve remission.

Assign this code when documentation confirms a diagnosis of immunoproliferative small intestinal disease (IPSID) and explicitly states that the patient has not achieved remission. This typically applies to patients undergoing initial treatment, those with refractory disease, or those who have relapsed and are not currently in remission.

• Ensure documentation clearly specifies "not having achieved remission" to differentiate from codes indicating remission.
• Do not use this code for other types of small intestinal lymphomas or benign immunoproliferative disorders.
• Sequence this code as a primary diagnosis when it is the reason for the encounter or hospitalization.