Immunodeficiency Flashcards
List the pattern recognition receptors (PRRs).
- Toll-like receptors (TLR’s)
- NOD-like receptoes (NLR’s)
- Rig-like receptors (RLR’s)
- C-type lectins (CLR’s)
- scavenger receptors
What types of antimicrobial peptides are involved in the innate immune response?
- defensins
- cathelin
- protegrin
- granulsyin
- histatin
- secretory leukoprotease inhibitor
- probiotics
Which cells are involved in the innate immune response?
- Macrophages, dendritic cells, NK cells, NK-T cells, neutrophils, eosinophils, mast cells, basophils, and epithelial cells
What is immunodeficiency?
- Clinical situations where the immune system is not effective enough to protect the body against infection
- Can occur at any age.
What might be the cause of immunodeficiency?
- Can be argued that infection is always the result of transient immunodeficiency.
- Usually secondary to the effects of external factors
- Some are primary immunodeficiencies caused by genetic defects in individual components of the immune system.
- The type of infection is a guide to underlying cause.
- Laboratory tests confirm.
What are the causes of secondary immunodeficiencies?
- Stress (release of endogenous steroids - glucocorticoids)
- Surgery/burns
- Malnutrition
- Trauma (release of steroids)
- Cancer – especially lymphoproliferative disease
- Immunosuppressive effect of drugs inc. cancer therapy
- Lymphocytes
- Neutrophils
What is the root cause of a primary immunodeficiency?
Genetic defect - i.e. lacking any protein involved in inflammation, innate/acquired immunity
What is chronic granulomatous disease?
- Inability to acidify phagosome or form reactive oxygen species - leads to deep-seated bacterial infections e.g.
- Osteomyelitis
- Pneumonia
- Swollen lymph nodes
- Ginigivitis
- Non-malignant granulomas
- Inflammatory bowel disease
What kind of infections do terminal complement pathway deficiencies make a person susceptible to?
Meningococcal
What are the features of B cell deficiency?
- Lead to different degrees of loss of antibody secretion.
- Usually leads to recurrent bacterial infection with pyogenic organisms.
- Usually diagnosed at around 1-2 years since maternal IgG protects.
- Treat with antibiotics then iv IgG For life.
- Most are very serious
- some less serious e.g IgA deficiency.
Why are T cell deficiencies usually more dramatic than B cell deficiencies?
- Usually more dramatic since B cells also need T cell help.
- Symptoms are recurrent infection with opportunistic infections, bacteria, viruses,
- Fungi (candida), protozoa (pneumocystis).
What is severe combined immunodeficiency disorder (SCID)?
- Defect in both B and T cells
What are the primary T cell deficiencies?
- Severe Combined Immunodeficiency syndromes
- Adenosine Deaminase Deficiency
- Purine Nucleoside Phosphorylase Deficiency
- MHC Class II Deficiency
- Wiskott-Aldrich Syndrome
What are the primary B cell deficiencies?
- Common Variable Immunodeficiency
- X-linked agammaglobulinaemia
- Autosomal recessive Hyper IgM syndrome
- IgA Deficiency
- IgG Subclass Deficiency
- Transient Hypogammaglobulinaemia of infancy
What procedure can provide a cure in SCID?
Bone marrow transplant
