D84.0

HCC Category Mapping

What This Code Means

A rare immune system disorder where white blood cells lack a specific protein (LFA-1) needed to fight infections and communicate with other immune cells.

Coding Tips

• •This is a specific defect in cell adhesion molecules; document the specific immune function impairment if present
• •May require additional codes for associated infections or complications

Clinical Significance

Lymphocyte function antigen-1 defect, also known as leukocyte adhesion deficiency type 1, is a rare autosomal recessive immunodeficiency caused by deficiency or dysfunction of the CD18 integrin subunit. This prevents neutrophils and other leukocytes from adhering to blood vessel walls and migrating to infection sites, resulting in severe recurrent bacterial and fungal infections with characteristically delayed umbilical cord separation and poor wound healing.

Documentation Requirements

• ✓Documentation must include flow cytometry demonstrating absent or reduced CD18/CD11a expression on leukocytes, persistent leukocytosis (white blood cell counts often exceeding 15,000 even without active infection), infection history with specific organisms, delayed cord separation history in infants, and genetic testing confirming ITGB2 gene mutation.
• ✓Document severity classification (severe with less than 2% expression vs.
• ✓moderate with 2-30%).

Commonly Confused Codes