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Immunodeficiency disorders

Published Sept. 14, 2020
• Reviewed by a UI Health Care Provider
on Sept. 1, 2020

Your immune system is made up of lymphoid tissue in the body. These are:

  • bone marrow
  • lymph nodes
  • thymus
  • tonsils
  • spleen
  • parts of your gastrointestinal tract
  • Proteins (called antibodies) and white blood cells in the blood are also part of your immune system.

Your immune system helps protects you from harmful germs such as bacteria, viruses, fungi,  and parasites. Your immune system makes proteins when it detects a germ. These antibodies and complement can kill the harmful germs. Your immune system can also make cells that eat the germs or kill host cells that have the germs. Immunodeficiency disorders happen when you do not have an immune response or it is weaker.

Causes

  • Many immunodeficiency disorders are inherited gene mutations.
  • The disorders that affect B cells are:
    • Hypogammaglobulinemia or Common Variable Immunodeficiency (CVID).
      • These can lead to respiratory and gastrointestinal bacterial infections.
    • Agammaglobulinemia.
      • You can get severe infections when you are young.
      • It can lead to death if not treated.
  • The disorders that affect T cells can cause you to have many yeast, mold or viral infections.
  • You can get inherited combined immunodeficiency.
    • This affects both T cells and B cells.
    • It can be deadly in your first year of life if it is not treated early.

Acquired immunodeficiency can be from other diseases or treatments. These are:

  • HIV/AIDS
  • Malnutrition
  • Cancer
  • Medicines such as immunosuppressants or chemotherapy.
  • Removal of the spleen.
  • Diabetes

Some syndromes can have immunodeficiency part of their disorder. These are:

  • Ataxia-telangiectasia
  • DiGeorge syndrome
  • Job’s or Hyper-IgE syndrome
  • Wiskott-Aldrich syndrome

Symptoms can be:

  • Repeated infections
  • Infection from germs that do not usually cause infection
  • Poor response to treatment for infections
  • Delayed or incomplete recovery from illness
  • Certain types of cancers (such as Kaposi sarcoma or non-Hodgkin lymphoma)

Exams and tests can be:

  • Complement levels
  • Immunoglobulin levels
  • Response to vaccines
  • Protein electrophoresis (blood or urine)
  • Lymphocyte count and activation
  • White blood cell count

Treatment

  • Do not be around people who have infections or contagious disorders.
  • Do not be near people who have been vaccinated with live virus vaccines within the past 2 weeks.
  • You many need antibiotics, antivirals, and/or antifungal drugs to treat infections or to keep them from coming back.
  • You should be vaccinated against certain bacteria if you had your spleen removed. These are:
    • Streptococcus pneumonia
    • Haemophilus influenzae
    • Neisseria meningitidis
  • You may need bone marrow transplants.
  • You may need immunoglobulin replacement infusions if you have low or no levels of some immunoglobulins.

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