Immunodeficiency associated with oth major defects

This code signifies a primary immunodeficiency disorder that occurs in conjunction with other significant congenital anomalies or systemic defects not classified elsewhere. It indicates that the patient's immune system dysfunction is part of a broader syndrome or condition involving multiple organ systems or developmental abnormalities.

Use this code when documentation clearly links an immunodeficiency to a specific, named syndrome or congenital defect that is not explicitly listed under other D80-D84 codes. This often applies to rare genetic syndromes where immunodeficiency is a known component, and the specific syndrome has its own ICD-10-CM code. Documentation should specify both the immunodeficiency and the associated major defect.

• Always code the underlying major defect or syndrome first, if known and applicable, followed by this code to specify the associated immunodeficiency.
• Distinguish from D82.0 (Wiskott-Aldrich syndrome) or D82.1 (DiGeorge syndrome), which have their own specific codes for immunodeficiency associated with particular major defects.
• Ensure the medical record explicitly states the association between the immunodeficiency and the "other specified major defect" rather than just co-occurrence.