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Hypogammaglobulinaemia


This guideline applies to adults

 

Introduction

Hypogammaglobulinemia may be:

Primary:

  • Usually diagnosed in infancy and managed by Paediatrics/ Immunologists but increasingly common to be picked up in adults.

Secondary:

  • Excessive loss of immunoglobulin e.g. protein losing enteropathy, nephrotic syndrome, severe burns
  • Drug induced e.g. chemotherapy, corticosteroids, immunosuppressants
  • Malignancy e.g. lymphoproliferative disorders, myeloma, lymphoma, Good’s syndrome
  • Autoimmune disease

 

Patients usually present with recurrent infections (especially sinopulmonary & GI infections) but also autoimmune or connective tissue disease.


In scope:  Hypogammaglobulinemia (low levels of IgG, IgA, IgM)


Not in scope:  All other forms of blood disorders.


  

Red Flag Features

  • B symptoms - weight loss, fevers, night sweats or bulky lymph nodes
  • Blood tests suggestive of malignancy

*This list is non exhaustive 


 

Investigations required prior to referral

  • FBC, U&Es, Bone profile, Protein Electrophoresis, Serum Free Light Chains (or urine for Bence -Jones protein)
  • If paraprotein or abnormal serum free light chain ratio present, please refer to MGUS/Myeloma guideline.
  • If lymphocytosis present, please refer to Lymphocytosis guideline


 

Management optimisation

The main principles of management include treatment of infections (consider starting antibiotics early in acute infections), treatment of any underlying cause and referral for consideration of immunoglobulin replacement. [1]


 

Advice and Guidance

Haematology A&G can be accessed via ERS: Haematology Advice & Guidance

This service should not be used for suspected fast track referrals.


 

Referral

Urgent referral criteria

If concern over malignancy refer via Fast Track Suspected Cancer Referrals

 

Routine referral criteria

Consider routine referral to haematology if cytopenia present, otherwise refer to Immunology at University Hospital Plymouth



 

Supporting Information

For professionals

 

References

Peninsula Cancer Alliance, Haematology Site Specific Group, Advice and Guidance for non-haematological clinicians, October 2024 

 

  1. patient.info Hypogammaglobulinaemia (Plasma Deficiency) | Doctor  May 2025

 

Page Review Information

Review date:

27 May 2025

Next review date:

27 May 2027

Clinical editor:

Dr Laura Lomas

Contributors:

Dr Michelle Furtado, Peninsular Cancer Alliance - Haematology Site-Specific Group (SSG)