Updated: 12/25/2021

IgA Deficiency

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  • Snapshot
    • A 22-year-old female with a history of celiac disease comes to the office for chronic watery diarrhea. She recently returned from a vacation in Mexico. On further chart review, she has had multiple episodes of sinus infections, pneumonia, and otitis media throughout her childhood. After her last pregnancy, she received a blood transfusion following severe post-partum hemorrhage and experienced an anaphylactic reaction. Physical exam reveals chronic eczema on the flexural surface of the arms. Stool analysis is positive for Giardia.
  • Introduction
    • Primary immunodeficiency with decreased levels of IgA
  • Epidemiology
    • Most common primary immunodeficiency
    • May be inherited (recessive and dominant forms identified)
    • May be drug-induced
    • Most common in individuals of European descent
    • Prevalence 1 in 500 persons
  • ETIOLOGY
    • Pathogenesis
      • cause is unknown
      • B-cells fail to differentiate into IgA producing plasma cells
      • impairs mucosal immunity, leading to sinopulmonary infections
    • Associated conditions
      • increased incidence of autoimmune diseases
      • celiac disease
      • atopy
        • allergies
        • atopic dermatitis
        • asthma
  • Presentation
    • Symptoms
      • most = asymptomatic
      • sinus and lung infections
        • usually Streptococcus pneumoniae and other encapsulated bacteria
      • GI infections
        • especially giardiasis
      • severe allergies
      • atopic dermatitis
      • anaphylaxis with exposure to blood products containing IgA
  • IMAGING
    • Radiography
      • to identify infections secondary to low IgA
  • STUDIES
    • Serology
      • ↓ IgA (< 7 mg/dL)
      • normal IgG and IgM
      • false positive β-hCG
  • Differential Diagnosis
    • Common variable immunodeficiency
    • IgG deficiency
    • Ataxia-telangiectasia (↓ IgA, IgG, and IgE)
    • Hyper-IgM syndrome (↓ IgA, IgG, IgE, and ↑ IgM)
      • normal B and T cell by flow cytometry
        • may have deficient expression of CD40L on activated T-helper cells
      • presents with
        • severe pyogenic infections early in life
        • chronic diarrhea
        • opportunistic infections, such as recurrent pneumonia
      • increased risk for
        • hyperviscosity syndrome
          • due to elevated levels of IgM
        • malignancies (HCC and carcinoid tumor)
  • DIAGNOSIS
    • Diagnosis based on clinical history
  • Treatment
    • Prevention
      • avoid blood transfusions unless treated with saline wash or from IgA deficient individual
    • Usually no specific treatment
    • Antibiotics as needed for infections
    • Immunizations
    • Blood transfusions
      • wash RBCs with saline or
      • obtain blood from IgA-deficient donor
  • Complications
    • Recurrent sinopulmonary infections
    • Diarrhea from Giardiasis
    • Fatal anaphylaxis
  • Prognosis
    • Typically very good
    • Some patients spontaneously develop normal IgA levels
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(M2.RH.15.4671) A 16-year-old boy is brought to the emergency department following a car accident in which he suffered multiple injuries. He is accompanied by his mother. She reports that his medical history is notable only for recurrent sinusitis and otitis as a child. He lost a significant amount of blood from the accident, and he is transfused two units of O-negative blood on arrival at the emergency department. Shortly thereafter, he complains of itching and increasing shortness of breath. He develops stridor. Which of the following could have prevented this reaction?

QID: 107170
1

Pre-transfusion acetaminophen

0%

(0/27)

2

Pre-transfusion diphenhydramine

4%

(1/27)

3

Administering type-specific blood

7%

(2/27)

4

Administering IVIG with transfusion

4%

(1/27)

5

Administering washed blood products

81%

(22/27)

M 6 D

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(M2.RH.14.93) An 18-year-old man presents to a rural emergency department after being stabbed multiple times. The patient's past medical history is notable for obesity, diabetes, chronic upper respiratory infections, a 10 pack-year smoking history, and heart failure. He is protecting his airway and he is oxygenating and ventilating well. His temperature is 97.6°F (36.4°C), blood pressure is 74/34 mmHg, pulse is 180/min, respirations are 24/min, and oxygen saturation is 98% on room air. The patient is started on whole blood and the surgeon on call is contacted to take the patient to the operating room. During the secondary survey, the patient complains of shortness of breath. His blood pressure is 54/14 mmHg, pulse is 200/min, respirations are 24/min, and oxygen saturation is 90% on room air. Physical exam is notable for bilateral wheezing on lung exam. The patient goes into cardiac arrest and after 30 minutes, attempts at resuscitation are terminated. Which of the following is associated with this patient's decompensation during resuscitation?

QID: 104884
1

Congenital long QT syndrome

51%

(23/45)

2

COPD

7%

(3/45)

3

Heart failure

13%

(6/45)

4

IgA deficiency

16%

(7/45)

5

Persistent intraabdominal bleeding

11%

(5/45)

M 6 E

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