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Immunodeficiency Disorders

Immunodeficiency disorders are conditions caused by abnormalities in the immune system, leading to recurrent and severe infections. They can be classified into primary (congenital) and secondary (acquired) types, with various underlying causes such as B-cell and T-cell deficiencies. Secondary immunodeficiencies can arise from factors like malnutrition, infections, and immunosuppressive therapies.

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10 views7 pages

Immunodeficiency Disorders

Immunodeficiency disorders are conditions caused by abnormalities in the immune system, leading to recurrent and severe infections. They can be classified into primary (congenital) and secondary (acquired) types, with various underlying causes such as B-cell and T-cell deficiencies. Secondary immunodeficiencies can arise from factors like malnutrition, infections, and immunosuppressive therapies.

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Immunodeficiency

Disorders
Dr. Md. Mehedi Hasan
Immunodeficiency
Immunodeficiency are clinical conditions that arise due to abnormality in the development
and functions of the immune system.

Cardinal symptoms of immunodeficiency:


Cardinal symptoms of immunodeficiency is infection, which is recurrent, severe, or
presenting in an unusual way.

Types of immunodeficiency disorders:


a. Primary / congenital immunodeficiency disorders
b. Secondary / acquired immunodeficiency disorders
Primary / Congenital
Immunodeficiency Disorders
The immunodeficient conditions that result before birth, arte called primary
immunodeficiencies. Primary immunodeficiency disorders may result from the following
conditions:
Types & causes:
1. B-cell deficiencies:
• X-linked hypogammaglobulinaemia (Bruton’s agammaglobulinaemia)
• Selective immunoglobulin deficiencies (e.g. deficiency of IgA, IgM, IgG)
2. T-cell deficiencies:
• Thymic aplasia (DiGeorge’s syndrome)
• Chronic mucocutaneous candidiasis
• Hyper-IgM syndrome
• Interleukin-12 receptor deficiency
3. Combined B-cell & T-cell deficiencies:
• Severe combined immunodeficiency disease (SCID)
• Wiskott-Aldrich syndrome
• Ataxia-Telangiectasia
Primary / Congenital
Immunodeficiency Disorders
Continued….
4. Complement deficiencies:
• Hereditary angioedema
• Recurrent infections
• Autoimmune diseases
• Paroxysmal nocturnal haemoglobinuria
5. Phagocyte deficiencies:
• Chronic granulomatous diseases (CGD)
• Chediac-Higashi syndrome
• Job’s syndrome (Hyper-IgE syndrome)
• Leukocyte adhesion deficiency syndrome
• Cyclic neutropenia
• Myeloperoxidase deficiency
• Interferon-Gamma receptor deficeiency
Secondary / Acquired
Immunodeficiency Disorders
The immunodeficient conditions that result after birth, arte called secondary / acquired
immunodeficiencies. Secondary immunodeficiency disorders may result from the following
conditions:
Types & causes:
1. B-cell deficiencies:
• Common variable hypogammaglobulinaemia
• Malnutrition
2. T-cell deficiencies:
• Acquired immunodeficiency syndrome (AIDS)
• Measles
3. Combined B-cell & T-cell deficiencies:
• Liver failure
• Malnutrion
4. Phagocyte deficiencies:
• Neutropenia
Secondary Immunodeficiency
Predisposing factors:
a. Skin disorders, e.g. burns, severe eczema
b. Malnutrition or malabsorption
c. Loss of protein, e.g. protein loosing enteropathy, nephritic syndrome etc.
d. Urinary catheterization
e. Intravenous cannulation
f. Artificial prosthesis
g. Antibiotic therapy
h. Cancers
i. Microbial infections, e.g. HIV infections, measles etc.
j. Immunosuppressive drugs, e.g. Steroids

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