18  Immunology and Defense Systems

18.1 Learning Objectives

By the end of this chapter, you should be able to:

  1. Describe the three lines of defense against pathogens and how they interact
  2. Distinguish between innate and adaptive immunity and their components
  3. Explain how the lymphatic system supports immune function
  4. Describe how B cells and T cells recognize antigens and mount immune responses
  5. Explain the mechanisms of immunological memory and vaccination
  6. Analyze how immune system malfunctions lead to autoimmune diseases, allergies, and immunodeficiencies
  7. Describe how the immune system interacts with other body systems
  8. Apply immunological principles to understand infectious diseases, cancer, and transplantation

18.2 Introduction

The immune system is the body’s defense network against pathogens, cancer cells, and other threats. This remarkable system can distinguish self from non-self, remember past infections, and mount targeted responses while maintaining tolerance to the body’s own tissues. Understanding immunology is essential for combating infectious diseases, developing vaccines, treating autoimmune disorders, and advancing cancer therapies. This chapter explores how the immune system protects us while maintaining the delicate balance required for health.


18.3 Overview of Host Defenses

18.3.1 Three Lines of Defense

First line: Physical and chemical barriers

  • Skin: Physical barrier, acidic pH, antimicrobial peptides
  • Mucous membranes: Mucus trapping, ciliary clearance
  • Chemical barriers: Stomach acid, lysozyme, defensins

Second line: Innate immunity

  • Cells: Phagocytes, natural killer cells
  • Proteins: Complement, interferons, acute phase proteins
  • Processes: Inflammation, fever

Third line: Adaptive immunity

  • Cells: B lymphocytes, T lymphocytes
  • Features: Specificity, memory, self-tolerance

18.3.2 Pathogens and Threats

Types of pathogens:

  • Bacteria: Prokaryotes (tuberculosis, strep throat)
  • Viruses: Require host cells (influenza, HIV)
  • Fungi: Eukaryotes (candida, athlete’s foot)
  • Parasites: Protozoa (malaria), helminths (tapeworms)
  • Prions: Misfolded proteins (mad cow disease)

Other threats: Cancer cells, transplanted tissues, allergens

18.3.3 Lymphatic System

Components:

  • Lymph: Fluid similar to plasma
  • Lymph vessels: Transport lymph
  • Lymph nodes: Filter lymph, contain immune cells
  • Spleen: Filters blood
  • Thymus: T cell maturation
  • Tonsils/adenoids: Protect respiratory/digestive entry
  • Peyer’s patches: In intestinal wall

Functions: Immune cell transport, antigen presentation, fluid balance


18.4 Innate Immunity

18.4.1 Characteristics

Present from birth: No prior exposure needed

Rapid response: Minutes to hours

No memory: Same response each time

Limited specificity: Recognizes patterns

18.4.2 Cellular Components

Phagocytes: Engulf and destroy pathogens

  • Neutrophils: Most abundant, first responders
  • Macrophages: Tissue-resident, antigen presentation
  • Dendritic cells: Best antigen presenters

Natural killer (NK) cells: Kill virus-infected and cancer cells

  • Mechanism: Release perforins and granzymes
  • Recognition: Missing self (lack of MHC I)

Mast cells: Release histamine in inflammation Basophils/Eosinophils: Attack parasites, allergic responses

18.4.3 Molecular Components

Complement system: >30 proteins that enhance immunity

  • Pathways: Classical, alternative, lectin
  • Functions: Opsonization, inflammation, membrane attack

Interferons: Antiviral proteins (α, β, γ)

Acute phase proteins: CRP, fibrinogen (increase during infection)

Cytokines: Chemical messengers (interleukins, chemokines)

18.4.4 Inflammatory Response

Cardinal signs: Redness, heat, swelling, pain, loss of function

Steps:

  1. Recognition: Pathogen-associated molecular patterns (PAMPs)
  2. Vasodilation: Increased blood flow
  3. Increased permeability: Leakage of fluid and proteins
  4. Phagocyte migration: Chemotaxis to site
  5. Phagocytosis: Engulfment and destruction
  6. Tissue repair: Resolution phase

Fever: Elevated body temperature

  • Benefits: Inhibits pathogen growth, enhances immune responses

  • Pyrogens: Substances that cause fever


18.5 Adaptive Immunity

18.5.1 Characteristics

Specificity: Recognizes specific antigens

Memory: Faster, stronger response upon re-exposure

Self-tolerance: Normally doesn’t attack self

Requires prior exposure: Initial response takes days

18.5.2 Antigens and Epitopes

Antigen: Substance that elicits immune response

Epitope: Specific part of antigen recognized

Hapten: Small molecule that becomes antigenic when attached to carrier

18.5.3 Major Histocompatibility Complex (MHC)

MHC class I: Present on all nucleated cells

  • Presents: Endogenous antigens (viral proteins, tumor antigens)
  • Recognized by: CD8+ T cells

MHC class II: Present on antigen-presenting cells (APCs)

  • Presents: Exogenous antigens (bacterial proteins)
  • Recognized by: CD4+ T cells

MHC polymorphism: Many alleles in population → diverse recognition

18.5.4 B Lymphocytes (Humoral Immunity)

Development: Bone marrow

Maturation: Bone marrow (mammals), bursa (birds)

Antigen receptor: B cell receptor (BCR) = membrane-bound antibody

Activation: 1. Antigen binding to BCR 2. T helper cell help (for protein antigens) 3. Differentiation: Into plasma cells and memory B cells

Antibody structure:

  • Heavy and light chains: Constant and variable regions
  • Fab: Antigen-binding fragment
  • Fc: Constant fragment (determines class)

Antibody classes:

  • IgM: First response, pentamer
  • IgG: Most abundant, crosses placenta
  • IgA: Secretions (mucus, milk)
  • IgE: Allergies, parasites
  • IgD: B cell receptor

Antibody functions:

  • Neutralization: Blocks binding sites
  • Opsonization: Enhances phagocytosis
  • Complement activation: Classical pathway
  • ADCC: Antibody-dependent cellular cytotoxicity

18.5.5 T Lymphocytes (Cell-mediated Immunity)

Development: Bone marrow

Maturation: Thymus (positive and negative selection)

Types:

  • Helper T cells (CD4+): Activate other cells (Th1, Th2, Th17, Treg)
  • Cytotoxic T cells (CD8+): Kill infected/cancer cells
  • Memory T cells: Long-lived protection
  • Regulatory T cells (Treg): Suppress immune responses

T cell receptor (TCR): Recognizes antigen + MHC

Co-receptors: CD4 (MHC II), CD8 (MHC I)

Activation:

  1. Antigen presentation by APC
  2. Co-stimulation (e.g., CD28-B7 interaction)
  3. Cytokine signals

Effector functions:

  • Cytotoxic T cells: Release perforin, granzymes, Fas ligand
  • Helper T cells: Release cytokines that activate other cells

18.5.6 Immune Response Dynamics

Primary response: First exposure, lag phase, IgM then IgG

Secondary response: Memory cells respond faster, higher affinity IgG

Clonal selection: Specific clones expand upon antigen encounter

Affinity maturation: Somatic hypermutation improves antibody affinity


18.6 Immunological Memory and Vaccination

18.6.1 Basis of Immunological Memory

Memory cells: Long-lived B and T cells

  • More numerous than naive cells
  • Lower activation threshold
  • Faster proliferation and differentiation

Characteristics of memory:

  • Specificity: Same antigen
  • Duration: Years to lifetime
  • Enhanced response: Higher magnitude, faster

18.6.2 Types of Immunity

Active immunity: Individual produces own immune response

  • Natural: Infection
  • Artificial: Vaccination

Passive immunity: Receiving pre-formed antibodies

  • Natural: Maternal antibodies (placenta, breast milk)
  • Artificial: Immune globulin, antivenom

18.6.3 Vaccination Principles

Goals: Induce memory without causing disease

Types of vaccines:

  • Live attenuated: Weakened pathogen (MMR, oral polio)
  • Inactivated/killed: Dead pathogen (influenza, polio injection)
  • Subunit: Parts of pathogen (hepatitis B, HPV)
  • Toxoid: Inactivated toxin (tetanus, diphtheria)
  • mRNA/DNA: Genetic material encoding antigen (COVID-19 mRNA vaccines)

Herd immunity: Protection of unvaccinated when enough population immune

Vaccination schedules: Based on immune system development, pathogen exposure risk


18.7 Immunological Disorders

18.7.1 Autoimmune Diseases

Failure of self-tolerance: Immune system attacks self

Mechanisms:

  • Molecular mimicry: Pathogen antigen resembles self
  • Release of sequestered antigens: From privileged sites
  • Polyclonal B cell activation: Non-specific stimulation
  • Genetic predisposition: HLA associations

Examples:

  • Type 1 diabetes: Anti-islet cell antibodies
  • Rheumatoid arthritis: Anti-IgG antibodies (rheumatoid factor)
  • Multiple sclerosis: Anti-myelin antibodies
  • Systemic lupus erythematosus: Anti-nuclear antibodies

18.7.2 Hypersensitivities (Allergies)

Type I (Immediate): IgE-mediated (anaphylaxis, hay fever)

  • Sensitization: IgE production to allergen
  • Re-exposure: Allergen cross-links IgE on mast cells → degranulation
  • Mediators: Histamine, leukotrienes, prostaglandins
  • Treatment: Antihistamines, epinephrine, desensitization

Type II (Cytotoxic): Antibody-mediated cell destruction (blood transfusion reactions)

Type III (Immune complex): Antigen-antibody complexes deposit in tissues (serum sickness)

Type IV (Delayed): T cell-mediated (contact dermatitis, TB test)

18.7.3 Immunodeficiencies

Primary (congenital): Genetic defects present at birth

  • Severe combined immunodeficiency (SCID): Defective T and B cells (“bubble boy” disease)
  • X-linked agammaglobulinemia: Defective B cell development
  • Chronic granulomatous disease: Defective phagocyte oxidative burst
  • DiGeorge syndrome: Thymic aplasia

Secondary (acquired): Caused by external factors

  • HIV/AIDS: CD4+ T cell depletion
  • Malnutrition: Protein-energy malnutrition impairs immunity
  • Medications: Chemotherapy, immunosuppressants
  • Aging: Thymic involution, decreased immune function

Treatment: Antibiotics, immunoglobulin replacement, stem cell transplantation, gene therapy


18.8 Immune System Interactions

18.8.1 Neuroendocrine-Immune Axis

Bidirectional communication:

  • Stress effects: Cortisol suppresses immune function
  • Sickness behavior: Cytokines induce fever, fatigue, social withdrawal
  • Psychoneuroimmunology: Mind-body connections in health and disease

Hormonal regulation:

  • Sex hormones: Estrogen enhances, testosterone suppresses immunity
  • Growth hormone/IGF-1: Enhance immune function
  • Melatonin: Modulates circadian rhythms of immune cells

18.8.2 Microbiome and Immunity

Gut microbiome: Trillions of microbes influence immune development

Functions:

  • Training: Educates immune system to distinguish pathogens from commensals
  • Competition: Commensals outcompete pathogens for resources
  • Metabolites: Short-chain fatty acids regulate immune responses
  • Barrier function: Maintains intestinal epithelial integrity

Dysbiosis: Imbalanced microbiome linked to autoimmune diseases, allergies

18.8.3 Cancer Immunology

Cancer immunosurveillance: Immune system detects and eliminates cancer cells

Evasion mechanisms:

  • Loss of antigenicity: Reduced MHC expression
  • Immunosuppression: Secretion of TGF-β, IL-10
  • Checkpoint expression: PD-L1 binds PD-1 on T cells, inhibiting them

Immunotherapies:

  • Checkpoint inhibitors: Anti-PD-1, anti-CTLA-4 antibodies
  • CAR-T cells: Engineered T cells with chimeric antigen receptors
  • Cancer vaccines: Stimulate immune response against tumor antigens

18.9 Transplantation Immunology

18.9.1 Histocompatibility

Major histocompatibility antigens: MHC proteins (HLA in humans)

Minor histocompatibility antigens: Other polymorphic proteins

ABO blood group antigens: Important in organ transplantation

18.9.2 Types of Transplants

Autograft: Self to self (skin grafts, bone marrow autotransplant)

Isograft: Genetically identical donor (identical twins)

Allograft: Same species, different genetics (most organ transplants)

Xenograft: Different species (pig heart valves, experimental)

18.9.3 Rejection Mechanisms

Hyperacute rejection: Minutes to hours, pre-existing antibodies

Acute rejection: Days to weeks, T cell-mediated

Chronic rejection: Months to years, vascular changes, fibrosis

18.9.4 Immunosuppression

Drugs:

  • Calcineurin inhibitors: Cyclosporine, tacrolimus
  • Antiproliferatives: Azathioprine, mycophenolate
  • mTOR inhibitors: Sirolimus, everolimus
  • Corticosteroids: Prednisone

Complications: Increased infection risk, cancer, drug toxicity

18.9.5 Graft-versus-Host Disease (GVHD)

Occurs when: Donor immune cells attack recipient tissues

Common in: Bone marrow transplantation

Prevention: HLA matching, T cell depletion, immunosuppression


18.10 Emerging Topics in Immunology

18.10.1 Systems Immunology

High-throughput approaches: Transcriptomics, proteomics, metabolomics

Single-cell analysis: Reveals heterogeneity within immune cell populations

Computational modeling: Predicts immune responses, vaccine efficacy

18.10.2 Trained Immunity

Innate immune memory: Epigenetic reprogramming of innate cells

Mechanisms: Metabolic changes, histone modifications

Implications: Vaccines that protect beyond specific pathogens

18.10.3 Precision Immunology

Personalized vaccines: Based on individual immune profiles

Biomarkers: Predict disease susceptibility, treatment response

Theragnostic approaches: Combine diagnosis and treatment

18.10.4 One Health Immunology

Zoonotic diseases: Understanding spillover from animals to humans

Environmental immunology: Effects of pollution, climate change

Planetary health: Global approaches to infectious diseases


18.11 Chapter Summary

18.11.1 Key Concepts

  1. Immune system protects against pathogens through layered defenses
  2. Innate immunity provides rapid, non-specific protection
  3. Adaptive immunity provides specific, memory-based protection
  4. B cells mediate humoral immunity via antibodies
  5. T cells mediate cellular immunity via direct killing and helper functions
  6. Immunological memory enables faster, stronger responses upon re-exposure
  7. Vaccination harnesses immunological memory to prevent disease
  8. Immunological disorders include autoimmune diseases, allergies, and immunodeficiencies
  9. Immune system interacts with nervous, endocrine systems and microbiome
  10. Immunology applications include cancer therapy, transplantation, and emerging fields

18.11.2 Three Lines of Defense

Line Components Response Time Specificity Memory
First Skin, mucous membranes, chemical barriers Immediate Non-specific None
Second Phagocytes, NK cells, complement, inflammation Minutes-hours Pattern recognition None
Third B cells, T cells, antibodies Days (first), hours (memory) Highly specific Yes

18.11.3 Immune Cell Types

Cell Type Origin Major Functions Key Features
Neutrophil Bone marrow Phagocytosis, first responder Most abundant, multilobed nucleus
Macrophage Monocyte (blood) Phagocytosis, antigen presentation, tissue repair Tissue-resident, versatile
Dendritic cell Bone marrow Antigen presentation (best APC) Many processes, link innate/adaptive
Natural killer cell Bone marrow Kill virus-infected/cancer cells No prior sensitization needed
B cell Bone marrow Antibody production, antigen presentation Surface immunoglobulin, memory cells
Helper T cell Thymus (via bone marrow) Activate other immune cells CD4+, multiple subsets (Th1, Th2, etc.)
Cytotoxic T cell Thymus (via bone marrow) Kill infected/cancer cells CD8+, perforin/granzyme release
Regulatory T cell Thymus (via bone marrow) Suppress immune responses CD4+CD25+FoxP3+, prevent autoimmunity

18.11.4 Antibody Classes

Class Structure Location Functions Special Features
IgM Pentamer Blood, lymph First response, complement activation Largest, 10 binding sites
IgG Monomer Blood, tissue fluids Main blood antibody, crosses placenta Most abundant, longest half-life
IgA Dimer Secretions (mucus, milk) Mucosal immunity, neutralization Secretory component protects from enzymes
IgE Monomer Bound to mast cells/basophils Allergies, parasite defense Lowest concentration, binds Fcε receptors
IgD Monomer B cell surface B cell receptor Function not fully understood

18.11.5 Hypersensitivity Types

Type Name Mechanism Time Course Examples
I Immediate (allergic) IgE, mast cell degranulation Minutes Anaphylaxis, hay fever, asthma
II Cytotoxic Antibody vs. cell surface antigens Hours-days Blood transfusion reactions, hemolytic disease
III Immune complex Antigen-antibody complexes deposit Days-weeks Serum sickness, lupus nephritis
IV Delayed T cell-mediated 2-3 days Contact dermatitis, TB test, graft rejection

18.11.6 Immunodeficiency Types

Type Cause Examples Key Features
Primary Genetic defects SCID, X-linked agammaglobulinemia Present from birth, often severe
Secondary External factors HIV/AIDS, malnutrition, drugs Acquired, may be reversible
Combined T and B cell defects SCID, Wiskott-Aldrich syndrome Severe infections, poor prognosis
Humoral B cell/antibody defects X-linked agammaglobulinemia, CVID Bacterial infections
Cellular T cell defects DiGeorge syndrome, HIV (late) Viral/fungal infections, cancer
Phagocytic Phagocyte defects Chronic granulomatous disease Bacterial/fungal infections, granulomas
Complement Complement defects C1-C9 deficiencies Bacterial infections, autoimmune-like

18.12 Review Questions

18.12.1 Level 1: Recall and Understanding

  1. List and describe the three lines of defense against pathogens.
  2. What are the main differences between innate and adaptive immunity?
  3. Describe the structure of an antibody and the functions of its different regions.
  4. What are the four types of hypersensitivity reactions, and what mechanisms underlie each?
  5. Explain the difference between active and passive immunity, giving examples of each.

18.12.2 Level 2: Application and Analysis

  1. A patient has recurrent bacterial infections but normal viral resistance. What type of immunodeficiency might this suggest, and why?
  2. Explain why a booster shot is often needed for vaccines, referring to immunological memory.
  3. How do cytotoxic T cells recognize and kill virus-infected cells?
  4. Compare and contrast the primary and secondary immune responses in terms of timing, antibody classes produced, and antibody affinity.
  5. Why might an immunosuppressed transplant recipient be at increased risk for certain cancers?

18.12.3 Level 3: Synthesis and Evaluation

  1. Design an experiment to determine whether a new vaccine candidate induces both humoral and cellular immunity.
  2. Evaluate the ethical considerations of using fetal tissues in vaccine development and testing.
  3. How has our understanding of checkpoint molecules in cancer immunology transformed cancer treatment?
  4. Propose a strategy for developing a universal flu vaccine that would provide protection against multiple strains.

18.13 Key Terms

  • Antigen: Substance that elicits an immune response
  • Antibody: Protein produced by B cells that binds specifically to antigens
  • Pathogen: Disease-causing organism
  • Phagocytosis: Engulfment and destruction of particles by cells
  • Inflammation: Local response to tissue damage or infection
  • Cytokine: Signaling protein that mediates immune responses
  • Major histocompatibility complex (MHC): Cell surface proteins that present antigens to T cells
  • B cell: Lymphocyte that produces antibodies
  • T cell: Lymphocyte that mediates cellular immunity
  • Immunological memory: Ability to mount faster, stronger response upon re-exposure
  • Vaccination: Administration of antigen to induce protective immunity
  • Autoimmunity: Immune response against self-antigens
  • Hypersensitivity: Excessive immune response causing tissue damage
  • Immunodeficiency: Impaired immune function
  • Tolerance: Lack of immune response to specific antigens
  • Complement: Group of plasma proteins that enhance immune responses

18.14 Further Reading

18.14.1 Books

  1. Janeway, C. A., Travers, P., Walport, M., & Shlomchik, M. J. (2001). Immunobiology: The Immune System in Health and Disease (5th ed.). Garland Science.
  2. Abbas, A. K., Lichtman, A. H., & Pillai, S. (2017). Cellular and Molecular Immunology (9th ed.). Elsevier.
  3. Murphy, K., & Weaver, C. (2016). Janeway’s Immunobiology (9th ed.). Garland Science.

18.14.2 Scientific Articles

  1. Burnet, F. M. (1957). A modification of Jerne’s theory of antibody production using the concept of clonal selection. Australian Journal of Science, 20, 67-69.
  2. Medzhitov, R., & Janeway, C. A. (1997). Innate immunity: The virtues of a nonclonal system of recognition. Cell, 91(3), 295-298.
  3. Allison, J. P. (2015). Immune checkpoint blockade in cancer therapy: The 2015 Lasker-DeBakey Clinical Medical Research Award. JAMA, 314(11), 1113-1114.

18.14.3 Online Resources

  1. The Immune System (NIH): https://www.niaid.nih.gov/research/immune-system
  2. Immunology Online: https://www.immunology.org
  3. Vaccine Education Center (CHOP): https://www.chop.edu/centers-programs/vaccine-education-center
  4. American Academy of Allergy, Asthma & Immunology: https://www.aaaai.org

18.15 Quantitative Problems

  1. Antibody-Antigen Binding: An antibody binds to its antigen with association constant Ka = 10⁸ M⁻¹.
    1. What is the dissociation constant Kd?
    2. If the antigen concentration is 10 nM, what fraction of antibody binding sites are occupied?
    3. What antigen concentration is needed for 90% occupancy?
    4. How does affinity maturation improve antibody binding (quantitatively)?
  2. Immune Cell Numbers: A human has approximately 5 liters of blood with 5 × 10⁹ white blood cells per liter.
    1. How many total white blood cells are in circulation?
    2. If 70% are neutrophils, 20% are lymphocytes, and 10% are others, how many of each?
    3. During an infection, neutrophil count increases 5-fold. What is the new total?
    4. Lymphocytes recirculate between blood and lymph. If they spend 30 minutes in blood per cycle, how many pass through blood per day?
  3. Vaccine Herd Immunity: Herd immunity threshold = 1 - 1/R₀, where R₀ is basic reproduction number. For measles, R₀ ≈ 15; for polio, R₀ ≈ 6; for COVID-19 (original strain), R₀ ≈ 3.
    1. Calculate herd immunity threshold for each disease.
    2. If vaccine efficacy is 95%, what vaccination coverage is needed?
    3. If a population of 10 million has 85% vaccination with 95% efficacy for measles, how many are susceptible?
    4. If one case is introduced, how many secondary cases would occur?

18.16 Case Study: HIV/AIDS

Background: HIV (human immunodeficiency virus) causes AIDS (acquired immunodeficiency syndrome) by destroying CD4+ T cells. Since its discovery in the 1980s, HIV has caused over 35 million deaths but is now manageable with antiretroviral therapy.

Questions:

  1. How does HIV enter and replicate in CD4+ T cells?
  2. Why does HIV specifically deplete CD4+ T cells, and what are the consequences?
  3. How do antiretroviral drugs target different stages of the HIV life cycle?
  4. Why has developing an HIV vaccine been so challenging?
  5. What are the prospects for an HIV cure (e.g., “Berlin patient,” gene editing)?

Data for analysis:

  • Transmission: Sexual contact, blood, mother-to-child
  • Progression: Acute infection → clinical latency → AIDS (without treatment)
  • Treatment: Combination antiretroviral therapy (cART) suppresses viral load
  • Prevention: PrEP (pre-exposure prophylaxis), PEP (post-exposure prophylaxis), condoms
  • Epidemiology: ~38 million people living with HIV worldwide (2021)

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