Déficit immunitaire commun variable

Common variable immunodeficiency is the most frequent symptomatic immunedeficiency of adulthood. It is defined by primary (opposite to secondary) hypogammaglobulinemia. It's an heterogeneous disorder, that can include repeated bacterial infections, and/or auto-immune and/or lymphoproliferative diseases. The diagnosis is suspected if infectious diseases are serious and/or unusually frequent, and it has to be confirmed by gammaglobulins and/or IgG, A and M dosage. Treatment is based on immunoglobulin replacement, which allow to drastically diminish the frequency and severity of infectious bacterial diseases. The pathophysiology of this rare disease is poorly understood, but the recent description of molecular defects in rare genetic diseases will maybe help us to understand the mechanisms of hypogammaglobulinemia in these patients.