D82.3

HCC Category Mapping

What This Code Means

A genetic immune disorder where the body cannot properly fight Epstein-Barr virus infections due to inherited immune system defects.

Coding Tips

• •Document evidence of EBV infection or exposure history in the medical record
• •This is a hereditary condition; consider coding family history if relevant to the encounter

Clinical Significance

Immunodeficiency following hereditary defective response to Epstein-Barr virus, also known as X-linked lymphoproliferative syndrome, is a rare genetic disorder where the immune system cannot mount an appropriate response to Epstein-Barr virus infection. This deficiency can result in fulminant infectious mononucleosis, hemophagocytic lymphohistiocytosis, dysgammaglobulinemia, and B-cell lymphoma upon initial Epstein-Barr virus exposure.

Documentation Requirements

• ✓Documentation must include genetic testing confirming SH2D1A (SAP) or XIAP gene mutation, Epstein-Barr virus serology and viral load, family history of fatal mononucleosis or lymphoma, natural killer cell and cytotoxic T-cell functional testing, and treatment plan including consideration of hematopoietic stem cell transplantation.
• ✓Document any history of hemophagocytic lymphohistiocytosis episodes or lymphoma.

Commonly Confused Codes