Notes on Immunodeficiency Diseases

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Defective Phagocyic Cells:

• CGD: NADPH oxidase deficiency: catalase+ bacterial and fungal infections.

• Chediak-Higashi syndrome: granule structural defect: recurrent bacterial infections.

• G6PD deficiency: lacks HMP shunt enzyme: catalase+ bacterial and fungal infections.

• Myeloperoxidase deficiency: granule enzyme deficiency: mild or none.

• Leukocyte adhesion deficiency: lacks CD18: recurrent chronic infections; failure to form pus.

Defects in Humoral Immunity:

• Bruton X-linked hypogammaglobulinemia: a tyrosin kinase deficiency; immature B cells. Treat with antibiotics.

• Transient hypogammaglobulinemia of infancy: delayed IgG synthesis. Treat with antibiotics.

• Common variable hypogammaglobulinemia: unknown. Treat with antibiotics.

• Selective IgA deficiency: lack IgA. Treat with antibiotics.

• X-linked hyper-IgM syndrome: lacks CD40L on T-cells. Treat with antibiotics and gammaglobulins.

Additional Readings:

Basic Immunology

1. Introduction to Immunology
2. Cells of Immunology
3. Selection of Lymphocytes
4. Primary Response to Antigen
5. Antigen Processing and Presentation
6. Humoral Effector Mechanism Generator
7. Cell-Mediated Effector Mechanism Generator
8. Vaccination and Immunotherapy
9. Immunodeficiency Diseases
10. Acquired Immunodeficiency Syndrome
11. Hypersensitivities and Autoimmunity Diseases
12. Immunology of Transplantation
13. Immunology of Cancer
14. Immunology Laboratory Technology
15. Acquired Immunity
16. Type II Hypersensitivity Reaction
17. Hypersensitivity Reactions
18. Primary Immunodeficiency
19. Secondary Immunodeficiency
20. Type III Hypersensitivity Reaction
21. Type IV Hypersensitivity Reaction
22. Type V Hypersensitivity Reaction
23. Tumor Immunology
24. Images of Antibodies
25. Th1 vs Th2 cells

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