Hereditary hypogammaglobulinemia

This code represents a primary immunodeficiency characterized by abnormally low levels of all types of immunoglobulins (antibodies) due to a genetic defect. Patients with this condition have impaired humoral immunity, making them highly susceptible to recurrent bacterial infections. It is a congenital disorder, meaning it is present from birth.

Use this code for patients diagnosed with a hereditary form of panhypogammaglobulinemia, such as X-linked agammaglobulinemia (Bruton's agammaglobulinemia). Documentation should clearly indicate a congenital or inherited etiology for the generalized low immunoglobulin levels, often supported by genetic testing or a family history of the disorder.

• Differentiate from secondary hypogammaglobulinemia (e.g., due to malignancy, medication side effects), which would be coded elsewhere.
• Ensure documentation specifies "hereditary" or "congenital" to support the use of this specific code.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter, especially for immunoglobulin replacement therapy.