D80.0

HCC Category Mapping

What This Code Means

A genetic condition where the body produces abnormally low levels of antibodies due to inherited factors.

Coding Tips

• •This code indicates a familial or hereditary pattern; confirm family history is documented
• •Distinguish from acquired hypogammaglobulinemia by verifying genetic/familial etiology

Clinical Significance

Hereditary hypogammaglobulinemia is a genetically determined primary immunodeficiency characterized by inadequate production of immunoglobulins, leaving patients highly susceptible to recurrent bacterial infections, particularly of the respiratory and gastrointestinal tracts. The most common form is X-linked agammaglobulinemia (Bruton disease), which presents in early childhood after maternal antibodies wane.

Documentation Requirements

• ✓Documentation must establish the hereditary/genetic basis through family history or genetic testing, quantitative immunoglobulin levels (Immunoglobulin G, Immunoglobulin A, Immunoglobulin M all significantly reduced), B-cell enumeration (typically absent or severely reduced in Bruton disease), and response to immunoglobulin replacement therapy.
• ✓Include documentation of recurrent infections and their frequency and severity.

Commonly Confused Codes