Hypogammaglobulinaemia

 

Hypogammaglobulinaemia may be:

Primary:

• Usually diagnosed in infancy and managed by Paediatrics/ Immunologists.

Secondary:

• Excessive  loss of immunoglobulin ( protein losing enteropathy, nephrotic syndrome, severe burns)
• Drug induced (chemotherapy, corticosteroids, immunosuppressants)
• Malignancy- CLL, myeloma ( light chain), lymphoma, Good’s syndrome

Patients usually present with recurrent infections (especially upper and lower respiratory tract infections & GI infections). There is also an increased incidence of autoimmune and connective tissue disorders.

 

Initial investigations:

FBC, Immunoglobulins

Consider U&E, LFTS, urine dipstick, serum electrophoresis/ urinary BJP

If hypogammaglobulinaemia is an incidental finding without obvious secondary cause, consider performing Free Serum Light Chains analysis and refer to the guidance on Myeloma.

 

Who to refer?

Refer to Haematology A&G if unexplained hypogammaglobulinaemia

 

 

References: https://bestpractice.bmj.com/topics/en-gb/1058

Contributors:

Dr Richard Noble, Consultant Haematologist, RCHT

Dr Bridgitte Wesson, GP & Kernow RMS Haematology guideline lead

 

Review date: 05/08/2020

Next Review 05/08/2021