Immunoglobulin A Deficiency
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Basics
Description
Serum IgA <7 mg/dL and a normal serum IgG and IgM in patients >4 years of age
Risk Factors
Genetics
- Exact pattern of inheritance remains unclear; however, the following associations may occur:
- 22q11 deletion syndrome
- 18q syndrome
- Partial deletions in the long or short arm and ring forms of chromosome 18
- Also associated with HLA-A1, HLA-A2, B8, DR3, DQ2 (8.1), and Dw3
- Also associated with non–MHC-associated genes involved in autoimmunity including IFIH1 on chromosome 2q24, CLEC16A on chromosome 16
Pathophysiology
- Failure of B lymphocyte differentiation into plasma cells producing IgA
- Block may be due to
- Defect in T-helper cells
- Antigen-presenting cells
- B cells
- Lack of effects from various cytokines, including IL-21, IL-4, IL-6, IL-7, or IL-10
Commonly Associated Conditions
Increased association with the following:
- Atopy
- Sinopulmonary infections
- GI infections (especially Giardia lamblia)
- Inflammatory bowel disease (Crohn disease and ulcerative colitis)
- Celiac disease
- Nodular lymphoid hyperplasia
- Malignancy
- Autoimmune illnesses
- Systemic lupus erythematosus
- Immune endocrinopathies (e.g., Graves disease, type 1 diabetes)
- Autoimmune hematologic conditions
- Chronic active hepatitis
-- To view the remaining sections of this topic, please log in or purchase a subscription --
Basics
Description
Serum IgA <7 mg/dL and a normal serum IgG and IgM in patients >4 years of age
Risk Factors
Genetics
- Exact pattern of inheritance remains unclear; however, the following associations may occur:
- 22q11 deletion syndrome
- 18q syndrome
- Partial deletions in the long or short arm and ring forms of chromosome 18
- Also associated with HLA-A1, HLA-A2, B8, DR3, DQ2 (8.1), and Dw3
- Also associated with non–MHC-associated genes involved in autoimmunity including IFIH1 on chromosome 2q24, CLEC16A on chromosome 16
Pathophysiology
- Failure of B lymphocyte differentiation into plasma cells producing IgA
- Block may be due to
- Defect in T-helper cells
- Antigen-presenting cells
- B cells
- Lack of effects from various cytokines, including IL-21, IL-4, IL-6, IL-7, or IL-10
Commonly Associated Conditions
Increased association with the following:
- Atopy
- Sinopulmonary infections
- GI infections (especially Giardia lamblia)
- Inflammatory bowel disease (Crohn disease and ulcerative colitis)
- Celiac disease
- Nodular lymphoid hyperplasia
- Malignancy
- Autoimmune illnesses
- Systemic lupus erythematosus
- Immune endocrinopathies (e.g., Graves disease, type 1 diabetes)
- Autoimmune hematologic conditions
- Chronic active hepatitis
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