Primary Immunodeficiency
Definition: Inborn errors affecting immune factors, leading to deficient immunity. |
Categories: Congenital immunodeficiency. Approximately 50% due to B cell issues. Around 30% linked to T cell defects. Roughly 18% are errors in phagocytes. Approximately 2% relate to complement deficiencies |
Secondary Immunodeficiency
Definition: Acquired immunodeficiencies, more common than primary immunodeficiencies. |
Causes: Age. Certain infectious agents. Medical interventions. Systemic disorders (e.g., diabetes, malnutrition, alcoholism, hepatitis). |
Autoimmune Disorders and Diagnosing
Autoimmunity: Immune system attacks healthy self-tissues. Genetics and exposure to certain infectious agents contribute. Over 100 autoimmune diseases described. |
Diagnosis: General signs and symptoms. Presentation varies based on affected tissues. Hypersensitivities are inappropriate immune responses. More common in developed nations (hygiene hypothesis). |
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Example of Primary Immunodeficiency
DiGeorge Syndrome |
Deletion in part of chromosome 22. Causes impaired thymus development. Impairs cellular immune responses. |
intervetions secondary Immunodeficiencies
Cancer treatments (e.g., radiation and chemotherapy). Steroid anti-inflammatory drugs (e.g., corticosteroids). Anti-seizure medications. |
Allergy and Type I Hypersensitivities
Allergy: Triggered by allergens, leading to IgE production. Immune system reacts to harmless substances. Examples: atopic asthma, atopic dermatitis. |
Systemic Anaphylaxis: Localized and systemic anaphylaxis. Systemic anaphylaxis is potentially life-threatening and treated with epinephrine. |
Diagnosis and Management: Diagnosis based on symptoms, blood/skin tests. Management includes allergen avoidance, medications, and desensitization immunotherapy. |
Type 4 Hypersensitivities/autoimmune
Type IV Hypersensitivities: T cell-mediated, not antibody-mediated. Manifest slowly, causing delayed hypersensitivity reactions. Responsible for autoimmune disorders. |
Autoimmune Type IV Hypersensitivities: Examples: Guillain-Barré syndrome, Hashimoto thyroiditis, Type I diabetes, multiple sclerosis, celiac disease. Therapies aim to reduce T cell response and inflammation. |
Nonautoimmune Type IV Hypersensitivities: Triggered by haptens. Examples: tuberculin skin test, contact dermatitis, transplant rejection, graft-versus-host disease. |
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Therapies for Primary Immunodeficiencies
Bone marrow transplants. Intravenous or subcutaneous antibody administration. Cytokine therapies. Experimental treatments (e.g., stem cell transplants, thymus transplantation, gene therapy). |
Immune Deficiencies and Cancer
The immune system protects against infections and cancer. |
Patients with compromised immune systems are at increased risk for certain cancers. |
Immunotherapies aim to boost immune defenses to prevent or treat cancer. |
Type II and Type III Hypersensitivities
Type II Hypersensitivities: IgG or IgM bind to cell surface or extracellular antigens, leading to complement activation and cell lysis. Examples: Goodpasture syndrome, autoimmune hemolytic anemia, rheumatic heart disease. |
Blood Groups and Transfusion Reactions: Blood types based on antigens on red blood cells. Incompatible transfusions cause hemolytic reactions. |
Type III Hypersensitivities: IgG or IgM antibodies bind to soluble targets, forming immune complexes. Examples: antivenoms, antitoxins. |
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