Immunoproliferative small intestinal disease, in remission

This code signifies a diagnosis of immunoproliferative small intestinal disease (IPSID), also known as alpha-heavy chain disease, that is currently in remission. IPSID is a rare form of non-Hodgkin lymphoma characterized by the proliferation of abnormal plasma cells in the small intestine, leading to malabsorption and other gastrointestinal symptoms. Remission indicates that the signs and symptoms of the disease have significantly decreased or disappeared.

This code is appropriate for patients with a documented history of IPSID who are currently asymptomatic and show no evidence of active disease based on clinical, endoscopic, and histological findings. Documentation should clearly state "in remission" or similar terminology following treatment. It should be used when the patient is being monitored for recurrence after successful therapy.

• Do not use this code for active IPSID; refer to C88.30 for unspecified or C88.39 for other specified sites.
• Ensure documentation explicitly states "in remission" to support the use of this subcategory.
• Consider sequencing this code after any codes for long-term drug therapy or follow-up examinations related to the remission status.