1. Organization of Immune System
2. Cells
2.1 Lymphocytes:
2.1.1 B-cells (immunoglobulins)
2.1.2 T-cells
2.2 Plasma cells (B-cells)
2.3 Macrophages/dendritic cells
2.4 Mast cells
2.5 Eosinophils (against parasites)
2.6 Natural Killer (NK) Cells
3. Important Molecules
3.1 Antigens
3.2 Antibodies
3.3 T-cell receptors
3.4 MHC molecules
3.5 Adhesion molecules
3.6 Cytokines
4. Immune Response
5. Non-specific (innate) Immunity
6. Specific Immunity
7. Categories of Immune Disorders
7.1 Hypersensitivity
7.1.1 Type I (anaphylaxis)
IgE (embedded in mast cell or basophils)
Mast cells release vasoactive substances
Preformed mediators
Secondary mediators:
Clinical manifestations of Type I Hypersensitivity
Systemic anaphylaxis (anaphylactic shock)
Local anaphylaxis (atopic allergy)
Asthma: (Complex mechanism/T-cells also involved)
7.1.2 Type II
Clinical examples:
Autoimmune hemolytic anemia
Goodpasture syndrome
Transfusion reactions
Grave's disease
Myasthenia gravis
7.1.3 Type III
Clinical examples:
Immune complex disease:
Serum sickness
Some types of vasculitis
Arthus reaction - experimental
Post-streptocccal glomerulonephritis
7.1.4 Type IV
Delayed-type cell-mediated
Clinical examples:
Tuberculosis
Contact dermatitis
Transplant rejection
Background
Major complications
Rejection
Infection
Graft-versus-host disease (GVHD):
Development of Cancers
Lymphoma
Kaposi sarcoma
Squamous cell CA-skin
Histocompatibility antigens
Class I antigens
Class II antigens
Phases of transplant rejection:
Hyperacute rejection
Acute rejection
Chronic rejection
7.1.5 Notes
7.2 Autoimmune Disease:
7.2.1 Etiology
7.2.2 Mechanisms
7.2.3 Organ-specific autoimmune disease
Vitiligo (skin hypopigmentation)
Thyroid:
Graves' disease
Hashimoto's disease
Addison's disease (autoimmune adrenalitis)
Pernicious anemia (autoimmune gastritis)
Hemolytic anemia
Myasthenia gravis
Diabetes mellitus type I
Primary biliary cirrhosis (anti-mitochondrial antibodies)
7.2.4 Non-organ specific autoimmune disease
Systemic Lupus Erythematosus (SLE)
Etiology
Pathogenesis
Pathology
Kidney: glomerulonephritis
Skin:
Joints:
Serositis:
Heart: nonbacterial verrucous endocarditis (Libman-Sacks)
Spleen: "onion-skin" pervivascular fibrosis
Lungs:
Clinical
Diagnosis
Systemic sclerosis (scleroderma)
Pathology
Pathogenesis
Clinical
Raynaud syndrome
Arthritis (similar to rheumatoid)
Dysphagia (fibrosis esophagus)
Pulmonary failure (lung fibrosis)
Malignant hypertension & renal failure
Cardiac arrhythmia & heart failure
Diffuse scleroderma
rapid progression
characteristic ANA
Inflammatory myopathies
polymyositis
dermatomyositis
CREST Syndrome
Female-male ratio 3:1
peak incidence 50 - 60
Clinical manifestations of CREST Syndrome:
Localized Scleroderma (Morphea)
Rheumatoid Disease
7.2.5 Mixed connective tissue disease
7.2.6 Sjogren syndrome
Pathology
Clinical
Sicca syndrome
Dry eyes
Dry mouth
Swollen parotid glands +/- (Mikulicz syndrome)
90% females
35 to 45 years
60% associated with other autoimmune disease (secondary Sjogren's syndrome):
7.2.7 Collagen-vascular diseases
Examples:
SLE
Rheumatoid disease
Systemic sclerosis
Mixed connective tissue disease
Sjogrens syndrome
7.3 Deficiency states/Immunodeficiency Syndromes
7.3.1 Primary immunodeficiencies
Primary (B-cell) antibody deficiency
Agammaglobulinemia of Bruton
Common variable immunodeficiency
Isolated IgA deficiency
Primary (T-cell) cell-mediated deficiency
DiGeorge syndrome
Immunodeficiency with thrombocytopenia (Wiskott-Aldrich syndrome)
Selective deficiency IgA or IgG
Ataxia-Telangiectasia Syndrome
Phagocyte deficiency (chronic granulomatous disease)
SCID (Severe Combined Immunodeficiency Disease)
Leukocyte Adhesion Deficiency
Chediak-Higashi Syndrome
7.3.2 Secondary immunodeficiencies
Infections
Antibody deficiency:
Cell-mediated deficiency:
Viruses
HIV/AIDS
Immune deficiency
Complications
Opportunistic infections
Risk groups
Increased risk for neoplasms
Natural history
Early acute phase
Middle phase
Final crisis phase
Full-blown AIDS
HIV infection
Non-transforming type C cytopathic retrovirus
Viral envelope gp 120 binds to receptors on (helper/CD4) T-cells
Reverse transcriptase allows virus to integrate into host cell genome
Transmission
Fungi
Bacteria
Protozoa (cryptosporidia)
Complement deficiency
Phagocyte deficiency:
7.3.3 Genetic deficiencies of complement system
7.3.4 Amyloidosis
Amyloid
Amyloid States
Immunocyte Dyscrasia-Associated (Primary) Amyloidosis
Reactive Systemic (Secondary) Amyloidosis
Heredofamilial Amyloidosis
Hemodialysis-Associated Amyloidosis
Localized Amyloidosis
Amyloid of Aging
Endocrine Amyloid
Pathology
8. Glossary of Terms
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IMMUNOPATHOLOGY
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