Hypogammaglobulinaemia is an immune disorder characterized by a reduction in all types of gammaglobulins.
While hypogammaglobulinaemia means some of loss of gammaglobulins, a total loss is termed agammaglobulinaemia which can occur in as an x linked form - X-linked agammaglobulinemia.
The most commonly recognized clinical feature is recurrent infection (often respiratory). Increased incidence of parasitic gastrointestinal infections may also be present.
It may be primary of acquired.
- primary hypogammaglobulinemia
- transient hypogammalglobulinaemia in infancy
- multiple myeloma
- chronic lymphocytic leukemia (CLL) -
- hypogammaglobulinemia is present in up to 85% of CLL patients
- primary amyloidosis
- thymic epithelial tumors - Good syndrome
- ~ 5% of patients with hypogammaglobulinaemia can have a thymoma
- ~ 10% of patients with a thymoma can have hypogammaglobulinaemia
- hypogammaglobulinaemia is part of common variable immunodeficiency (CVID)
There are decreased levels of immunoglobulins (especially IgG)
Features secondary to recurrent bronchopulmonary and sinus infections are common
- bronchiectasis - especially lower lobe bronchiectasis
- areas of atelectasis
- lung cysts
- mediastinal lymphadenopathy
- features of opportunistic infection