Background:  Immunoglobulins are antibodies. Five types exist: IgG, IgA, IgM, IgE and IgD 

Hypogammaglobulinaemia is low immunoglobulins. It may be either primary or secondary.

Primary hypogammaglobulinaemia 
  • 2 main types
    • Common variable immune deficiency 
      • Characterized by frequent infections (sinusitis, bronchitis, pneumonia) with encapsulated organisms (such as strep pneumoniae, haemophilus influenza)
      • There are reduced concentrations of all IGs and IgG levels must be more than 2 SD below the mean 
      • Between 25 and 48% of patients have splenomegaly 
      • Up to 50% of patients have transient or persistent diarrheoa giardia lamblia is the most commonly identified organism 
      • Associated with increased risk of 
        • Non-Hodgkin lymphoma (up to 8% of patients)
        • Gastric cancer 
        • Thymoma (known as Good syndrome when associated with hypogammaglobulinaemia) 
        • Systemic granulomatous disease 
      • Associated with 
        • SLE 
        • ITP 
        • AIHA 
        • Juvenile rheumatoid arthritis 
      • Treatment is monthly IVIG infusions 
    • Selective IgA deficiency 
      • Inherited in an autosomal dominant pattern with variable penetrance
      • Low IgA but normal other IGs 
      • Commonest immune deficiency – 1 in 875 in the UK
      • Most patients (85-90%) have no symptoms; some may have recurrent infections 
      • Management is to treat infections as they occur 

Secondary hypogammaglobulinaemia 
  • May occur due to
    • Nephrotic syndrome
    • Protein-losing enteropathies 
    • Some malignancies
    • Medications 
      • Phenytoin 
      • Carbamazepine
      • Sulfasalazine 
      • Gold 
      • Systemic steroids  
    • High-stress states 


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