Dysgammaglobulinaemia – Causes, Symptoms and Treatment
Dysgammaglobulinaemia is a disorder in which the balance of gammaglobulins in the blood is disrupted, with certain immunoglobulin classes being elevated or reduced. This can significantly impair immune defence.
Things worth knowing about "Dysgammaglobulinaemia"
Dysgammaglobulinaemia is a disorder in which the balance of gammaglobulins in the blood is disrupted, with certain immunoglobulin classes being elevated or reduced. This can significantly impair immune defence.
What is Dysgammaglobulinaemia?
Dysgammaglobulinaemia is an immune system disorder characterised by an imbalance in gammaglobulins – a group of serum proteins that includes antibodies (immunoglobulins). Unlike agammaglobulinaemia, where all immunoglobulins are absent, dysgammaglobulinaemia affects only specific immunoglobulin classes: some may be deficient while others remain normal or are even elevated. The main immunoglobulin classes are IgA, IgG, IgM, IgD, and IgE.
Causes
Dysgammaglobulinaemia can be congenital (primary) or acquired (secondary):
- Primary forms: Genetic defects in B-lymphocyte development or function lead to a selective deficiency in certain immunoglobulin classes. A well-known example is selective IgA deficiency, the most common primary antibody deficiency.
- Secondary forms: These arise as a consequence of other diseases or treatments, such as malignant lymphomas, multiple myeloma, chronic infections, autoimmune diseases, or as a side effect of immunosuppressants and cytostatic drugs.
Symptoms
The clinical presentation depends on which immunoglobulin class(es) are affected and to what degree. Common symptoms include:
- Increased susceptibility to bacterial, viral, or fungal infections, which often recur or follow an unusually severe course
- Chronic respiratory tract infections (sinusitis, bronchitis, pneumonia)
- Gastrointestinal complaints and diarrhoea, especially in IgA deficiency
- Autoimmune phenomena such as rheumatoid arthritis or autoimmune cytopenia
- In some cases, the condition may be asymptomatic if the affected classes do not play a critical protective role
Diagnosis
Diagnosis is established through blood laboratory tests:
- Serum protein electrophoresis: Provides an initial overview of the distribution of protein fractions in the blood serum
- Immunoglobulin quantification: Direct measurement of individual immunoglobulin classes IgA, IgG, IgM, IgD, and IgE
- Flow cytometry: Analysis of B-cell subpopulations to differentiate the underlying cause
- Genetic testing: In cases of suspected primary immunodeficiency, to identify specific gene mutations
- Clinical history and physical examination to assess infection history and comorbidities
Treatment
Treatment is guided by the underlying cause and clinical severity:
- Immunoglobulin replacement therapy (IVIG/SCIG): Intravenous or subcutaneous administration of immunoglobulins, particularly in pronounced IgG deficiency with recurrent infections
- Antibiotic prophylaxis: Long-term antibiotic treatment for infection prevention in selected patients
- Treatment of the underlying condition: In secondary forms, treating the causative disease (e.g., chemotherapy for lymphomas) is the primary approach
- Vaccinations: Where applicable, to prevent specific infections; live vaccines are contraindicated in severe immunodeficiency
- Regular immunological follow-up to monitor disease progression
References
- Cunningham-Rundles C. - Physiology of IgA and IgA deficiency. Journal of Clinical Immunology, 21(5):303-309, 2001.
- World Health Organization (WHO) - Primary Immunodeficiency Diseases. Report of an IUIS Scientific Committee. Clinical and Experimental Immunology, 118 Suppl 1:1-28, 1999.
- Chapel H. et al. - Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood, 112(2):277-286, 2008.
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