- A 7-year-old patient presents to the emergency room after a motor vehicle accident, in which she loses a lot of blood. She is immediately transfused with blood. However, she develops difficulty breathing and oral-facial swelling. Physical exam reveals expiratory wheezes. She is immediately given epinephrine. On further questioning, physical exam reveals atopic dermatitis and recurrent ear and lung infections.
- Primary immunodeficiency with decreased levels of IgA
- most common primary immunodeficiency
- B-cells fail to differentiate into IgA-producing plasma cells
- unknown cause
- impairs mucosal immunity, leading to sinopulmonary infections
- Associated conditions
- increased incidence of autoimmune diseases
- celiac disease
- atopic dermatitis
- most = asymptomatic
- sinus and lung infections
- GI infections
- anaphylaxis with exposure to blood products with IgA
- Diagnosis based on clinical history
- ↓ IgA (< 7 mg/dL)
- normal IgG, IgM, and IgG
- false positive β-HCG
- Common variable immunodeficiency
- IgG deficiency
- Ataxia-telangiectasia (↓ IgA, IgG, and IgE)
- Hyper-IgM syndrome (↓ IgA, IgG, IgE, and ↑ IgM)
- Usually no specific treatment
- Antibiotics as needed for infections
- Blood transfusions
- obtain blood from IgA-deficient donor
|Prognosis, Prevention, and Complications
- typically very good
- some patients spontaneously develop normal IgA levels
- avoid blood transfusions unless from IgA-deficient individual
- recurrent sinopulmonary infections
- diarrhea from giardiasis
- fatal anaphylaxis
AVERAGE 4.0 of 5 RATINGS
Qbank (0 Questions)
Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions,
Personal Observations, and Biomechanic Studies)
Cooper MA, Pommering TL, Korányi K. Primary immunodeficiencies. Am Fam Physician. 2003 Nov 15;68(10):2001-8.
PMID:14655810 (Link to Abstract)
Yel L. Selective IgA deficiency. J Clin Immunol. 2010 Jan;30(1):10-6. doi: 10.1007/s10875-009-9357-x. Epub 2010 Jan 26. Review.
PMID:20101521 (Link to Abstract)
- First Aid for the USMLE STEP 1. Le, Tao. New York: McGraw-Hill Medical, 2013.
- Rapid Review Pathology Revised Reprint. Goljan, Edward. New York: Mosby, 2011.
- Clinical Microbiology Made Ridiculously Simple 5th Ed. Gladwin, Mark. Miami: MedMaster Inc, 2011.
- medEssentials for the USMLE Step 1. Manley, Michael. New York: Kaplan Publishing, 2012.
- Katzung & Trevnor's Pharmacology Examination and Board Review. Trevor, Anthony. New York: Lange Medical Books, 2012.