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5 out of 9

The commonest primary immunodeficiency is (AIPG 2010)

A Common variable immunodeficiency

B Isolated IgA immunodeficiency

C Wiskott-Aldrich syndrome


Ans. B i.e., Isolated IgA immunodeficiency [Ref Harrison 18th /e p. 2704]

"Selective deficiency of IgA is the most common immunodeficiency, occurring in about 1 in 1000 individuals".

a. Chandrasoma Taylor

i. "An inability to produce antibodies up the IgA1 and IgA2 subclasses occurs in approximately 1 in 600 individuals, a much higher incidence than is seen for other primary immunodeficiency". - Harrison

b. IgA deficiency

i. IgA deficiency is often familial

ii. It may be acquired by :

Congenital infections such as toxoplasmosis, rubella, CMV

Treatment with phenytoin

c. Pathogenesis

i. Block in B cell differentiation due to defective interaction between T and B cells. Naive B cells are not able to differentiate into IgA - producing cells.

d. Features

i. Respiratory infections

ii. Chronic diarrheal diseases

iii. Increased incidence of asthma and other atopic disease

iv. Frequent production of autoantibodies

v. Association with arthritis and SLE

vi. Reduction of IgG2 and IgG4

e. Common variable immunodeficiency

The feature common to all patients is hypogammaglobulinemia generally affecting all the antibody classes but sometimes only IgG.

f. Pathogenesis

i. Patients have normal numbers of B cells.

ii. B cells are not able to differentiate into plasma cells.

g. Features

i. Recurrent sinopulmonary infections

ii. Recurrent herpesvirus infection

iii. Persistent diarrhea caused by G. lamblia

iv. Meningoencephalitis caused by enterovirus.

v. High incidence of autoimmune diseases e.g. R.

vi. Increased risk of lymphoid malignancy and gastric cancer, particularly in women.

vii. Relatives of these patients have high incidence of selective IgA deficiency.

viii. T/t I. V. immunoglobulin

Note: AIDS is a type of secondary immunodeficiency (not primary).

Immunopathology Flashcard List

9 flashcards