D82.0

HCC Category Mapping

What This Code Means

A rare inherited disorder affecting both the immune system and blood cells, causing recurrent infections, bleeding problems, and eczema.

Coding Tips

• •This syndrome has multiple manifestations; document immune deficiency, thrombocytopenia, and eczema separately if present
• •Often requires documentation of specific infections and bleeding episodes for comprehensive clinical picture

Clinical Significance

Wiskott-Aldrich syndrome is an X-linked recessive primary immunodeficiency characterized by the classic triad of thrombocytopenia with small platelets, eczema, and recurrent infections due to combined T-cell and B-cell dysfunction. The condition results from mutations in the WASP gene and carries increased risk of autoimmune disease and lymphoid malignancies, particularly in adolescence and young adulthood.

Documentation Requirements

• ✓Documentation must include the clinical triad components (thrombocytopenia with characteristically small platelet volume, eczema severity, infection history), WASP gene mutation confirmation, complete blood count showing thrombocytopenia with low mean platelet volume, immunoglobulin levels (typically low Immunoglobulin M with elevated Immunoglobulin A and Immunoglobulin E), and treatment plan including consideration of hematopoietic stem cell transplantation.

Commonly Confused Codes