hypogammaglobulinemia

Hypogammaglobulinaemia is an immune disorder characterized by a reduction in all types of gammaglobulins.

Terminology

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.

Clinical presentation

The most commonly recognized clinical feature is recurrent infection (often respiratory). Increased incidence of parasitic gastrointestinal infections may also be present.

Pathology

Causes

It may be primary of acquired.

Primary

• primary hypogammaglobulinemia 
• transient hypogammalglobulinaemia in infancy

Acquired

• multiple myeloma
• chronic lymphocytic leukemia (CLL) - 
  • hypogammaglobulinemia is present in up to 85% of CLL patients
• primary amyloidosis
• medications
  • rituximab

Other associations

• 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)

Serology

There are decreased levels of immunoglobulins (especially IgG)

Radiographic features

Features secondary to recurrent bronchopulmonary and sinus infections are common

These include

Thorax

• bronchiectasis - especially lower lobe bronchiectasis
• areas of atelectasis
• lung cysts
• mediastinal lymphadenopathy
• features of opportunistic infection
  • P jiroveci pneumonia
  • aspergillus infection
    • aspergilloma

Abdomen

• splenomegaly

Siehe auch:

• Agammaglobulinämie

und weiter:

• Thymom
• invasives Thymom