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 lineage

T

lymphocyte

lineage

Bone

marrow

Thymus

Immature

B lymphocytes

Mature, naive

B lymphocytes

Naive

T lymphocytes Naive

T lymphocytes

Lymph

nodes

Spleen

Mucosal and

cutaneous

lymphoid

tissues

Recirculation

Recirculation

Generative

lymphoid

organs

Peripheral

(secondary)

lymphoid organs

Blood,

lymph

Common

lymphoid

precursor

Bone

Marrow

Fig. 1.10 Maturation and tissue distribution of lymphocytes. Lymphocytes develop from precursors in

the generative lymphoid organs (bone marrow and thymus). Mature lymphocytes enter the peripheral lymphoid organs, where they respond to foreign antigens and recirculate in the blood and lymph. Some immature

B cells leave the bone marrow and complete their maturation in the spleen (not shown).

CHAPTER 1 Introduction to the Immune System 13

Cell type Stage

B lymphocytes

T lymphocytes

T lymphocytes

B lymphocytes

Migration Preferentially

to peripheral

lymph nodes

Preferentially to

inflamed tissues

Heterogenous: different

subsets to lymph nodes,

mucosa and other tissues

Very low High Low

None Cytokine secretion;

cytotoxic activity

None

Frequency of cells

responsive to

particular antigen

Effector functions

A

B

Antigen

recognition

Antigen

recognition

Proliferation

Proliferation

Differentiation

Differentiation

Naive cell Memory lymphocyte Activated or

effector lymphocyte

Naive cell Memory

lymphocyte

Activated or

effector lymphocyte

Membrane

immunoglobulin

(Ig) isotype

Affinity of

Ig produced

IgM and IgD Frequently IgG,

IgA, and IgE (low level

in plasma cells)

Frequently IgG,

IgA, and IgE

Relatively low Increases during

immune response

Relatively high

Effector functions None Antibody secretion None

Fig. 1.11 Stages in the life history of lymphocytes. A, Naive lymphocytes recognize foreign antigens to initiate

adaptive immune responses. Naive lymphocytes need signals in addition to antigens to proliferate and differentiate into effector cells; these additional signals are not shown. Effector cells, which develop from naive cells,

function to eliminate antigens. The effector cells of the B lymphocyte lineage are antibody-secreting plasma cells

(some of which are long lived). The effector cells of the CD4 T lymphocyte lineage produce cytokines. (The effector cells of the CD8 lineage are CTLs; these are not shown.) Other progeny of the antigen-stimulated lymphocytes

differentiate into long-lived memory cells. B, The important characteristics of naive, effector, and memory cells

in the B and T lymphocyte lineages are summarized. The generation and functions of effector cells, including

changes in migration patterns and types of immunoglobulin produced, are described in later chapters.

14 CHAPTER 1 Introduction to the Immune System

lymphoid organs, where they may stay and produce

antibodies. Small numbers of antibody-secreting cells

are also found in the blood; these are called plasmablasts. Some of these migrate to the bone marrow,

where they mature into long-lived plasma cells and

continue to produce antibody years after the infection is eradicated, providing immediate protection in

case the infection recurs.

Effector CD4+ T cells (helper T cells) produce

proteins called cytokines that activate B cells, macrophages, and other cell types, thereby mediating the

helper function of this lineage. The properties of cytokines are listed in Appendix II and will be discussed in

later chapters. Effector CD8+ T cells (CTLs) have the

machinery to kill infected host cells. The development

and functions of these effector cells are also discussed

in later chapters. Effector T lymphocytes are short

lived and die as the antigen is eliminated.

• Memory cells, also generated from the progeny of

antigen-stimulated lymphocytes, can survive for long

periods in the absence of antigen. Therefore the frequency of memory cells increases with age, presumably because of exposure to environmental microbes.

In fact, memory cells make up less than 5% of peripheral blood T cells in a newborn but 50% or more in

an adult (Fig. 1.12). As individuals age, the gradual

accumulation of memory cells compensates for the

reduced output of new, naive T cells from the thymus,

which involutes after puberty (see Chapter 4). Memory cells are functionally inactive; they do not perform effector functions unless stimulated by antigen.

When memory cells encounter the same antigen that

induced their development, the cells rapidly respond

to initiate secondary immune responses. The signals

that generate and maintain memory cells are not well

understood but include cytokines.

Antigen-Presenting Cells

The common portals of entry for microbes—the skin

and gastrointestinal, respiratory, and genitourinary

tracts—contain specialized cells located in the epithelium that capture antigens, transport them to peripheral lymphoid tissues, and display (present) them to

lymphocytes. These are the first steps in the development of adaptive immune responses against antigens.

This function of antigen capture and presentation is

best understood for dendritic cells, the most specialized antigen-presenting cells (APCs) in the immune

system. Dendritic cells capture protein antigens of

microbes that cross epithelial barriers and transport

these antigens to regional lymph nodes, where they

display fragments of the proteins for recognition by T

lymphocytes. If a microbe has invaded through the epithelium, it may be phagocytosed and presented by tissue macrophages. Microbes or their antigens that enter

lymphoid organs may be captured by dendritic cells or

macrophages that reside in these organs and presented

to lymphocytes. The process of antigen presentation to

T cells is described in Chapter 3.

Dendritic cells have another important feature that

gives them the ability to stimulate T cell responses. These

specialized cells respond to microbes by producing surface proteins, called costimulators, which are required,

together with antigen, to activate naive T lymphocytes to

proliferate and differentiate into effector cells. Dendritic

cells express higher levels of these costimulatory proteins

than do other cell types and are thus the most potent

stimulators of naive T cells and the most efficient initiators of T cell responses. Other antigen-presentingcells,

such as macrophages and B cells, present antigens to differentiated effector T cells in various immune responses.

B lymphocytes may directly recognize the antigens of microbes (either released or on the surface of

the microbes), and macrophages and dendritic cells in

peripheral lymphoid organs may also capture antigens

and display them to B cells. A distinct type of cell called

% Blood T cells

Age (Years)

0

0

20

40

60

80

100

30 40 50 60 70 80

Naive T cells

Memory T cells

Thymic output

10 20

Fig. 1.12 Change in proportions of naive and memory

T cells with age. The proportions of naive and memory T

cells are based on data from multiple healthy individuals. The

estimate of thymic output is an approximation. (Courtesy Dr.

Donna L. Farber, Columbia University College of Physicians and

Surgeons, New York, NY.)

CHAPTER 1 Introduction to the Immune System 15

the follicular dendritic cell (FDC) resides in the germinal centers of lymphoid follicles in the peripheral lymphoid organs and displays antigens that stimulate the

differentiation of B cells in the follicles (see Chapter 7).

FDCs do not present antigens to T cells and differ from

the dendritic cells described earlier that function as

APCs for T lymphocytes.

TISSUES OF THE IMMUNE SYSTEM

The tissues of the immune system consist of the generative lymphoid organs, in which T and B lymphocytes mature and become competent to respond to

antigens, and the peripheral lymphoid organs, in

which adaptive immune responses to microbes are

initiated (see Fig. 1.10). Most of the lymphocytes in

a healthy human are found in lymphoid organs and

other tissues (Fig. 1.13). However, as we discuss later,

lymphocytes are unique among the cells of the body

because of their ability to recirculate, repeatedly going

through the blood to visit every secondary lymphoid

organ in the body. The generative (also called primary

or central) lymphoid organs are described in Chapter

4, when we discuss the process of lymphocyte maturation. The following section highlights some of the

features of peripheral (or secondary) lymphoid organs

that are important for the development of adaptive

immunity.

Peripheral (Secondary) Lymphoid Organs and

Tissues

The peripheral lymphoid organs and tissues, which

consist of the lymph nodes, the spleen, and the mucosal and cutaneous immune systems, are organized

in a way that promotes the development of adaptive

immune responses. T and B lymphocytes must locate

microbes that enter at any site in the body, then respond to

these microbes and eliminate them. The anatomic organization of peripheral lymphoid organs enables APCs to

concentrate antigens in these organs and lymphocytes to

locate and respond to the antigens. This organization is

complemented by a remarkable ability of lymphocytes to

circulate throughout the body in such a way that naive

lymphocytes preferentially go to the peripheral lymphoid

organs and tissues, in which antigen is concentrated,

whereas most effector cells go to sites of infection where

microbes must be eliminated. Furthermore, different

types of lymphocytes often need to communicate to generate effective immune responses. For example, within

peripheral lymphoid organs, helper T cells specific for an

antigen interact with and help B lymphocytes specific for

the same antigen, resulting in antibody production. An

important function of lymphoid organs is to bring these

rare cells together after stimulation by antigen so they

interact when they need to.

The major peripheral lymphoid organs share many

characteristics but also have some unique features.

• Lymph nodes are encapsulated nodular aggregates

of lymphoid tissues located along lymphatic channels

throughout the body (Fig. 1.14). Fluid constantly

leaks out of small blood vessels in all epithelia and

connective tissues and most parenchymal organs.

This fluid, called lymph, is drained by lymphatic vessels from the tissues to the lymph nodes and eventually back into the blood circulation. Therefore the

lymph contains a mixture of substances absorbed

from epithelia and tissues. As the lymph passes

through lymph nodes, APCs in the nodes are able

to sample the antigens of microbes that may enter

through epithelia into tissues. In addition, dendritic

cells pick up antigens of microbes from epithelia

and other tissues and transport these antigens to

the lymph nodes. The net result of these processes

of antigen capture and transport is that the antigens

of microbes entering through epithelia or colonizing tissues become concentrated in draining lymph

nodes.

Tissue Number of

lymphocytes

Spleen 70 x 109

50 x 109

20 x 109

10 x 109

190 x 109 Lymph nodes

Bone marrow

10 x 109 Blood

Skin

50 x 109 Intestines

Liver

30 x 109 Lungs

Fig. 1.13 Distribution of lymphocytes in lymphoid organs

and other tissues. Approximate numbers of lymphocytes in

different organs of healthy adults are shown.

16 CHAPTER 1 Introduction to the Immune System

• The spleen is a highly vascularized abdominal organ

that serves the same role in immune responses to

blood-borne antigens as that of lymph nodes in

responses to lymph-borne antigens (Fig. 1.15).

Blood entering the spleen flows through a network

of channels (sinusoids). Blood-borne antigens are

captured and concentrated by dendritic cells and

macrophages in the spleen. The spleen contains

abundant phagocytes that line the sinusoids, which

ingest and destroy microbes in the blood. These

macrophages also ingest and destroy old red blood

cells.

B cell zone

(follicle)

Afferent

lymphatic

vessel

Trabecula

Capsule

Vein

Artery Efferent

lymphatic

vessel

Medulla

T cell

zone

Germinal

center Medullary

sinus

Primary lymphoid

follicle (B cell zone)

Secondary

follicle with

germinal

center

Parafollicular

cortex (T cell zone)

Antigen

Lymphocytes

Subcapsular

sinus

High

endothelial

venule (HEV)

A

B

Fig. 1.14 Morphology of lymph nodes. A, Schematic diagram shows the structural organization of a lymph node.

B, Light micrograph shows a cross section of a lymph node

with numerous follicles in the cortex, some of which contain

lightly stained central areas (germinal centers).

Periarteriolar

lymphoid

sheath (PALS)

Germinal

center of

lymphoid

follicle

Periarteriolar

lymphoid

sheath (PALS)

B

Germinal

center of

lymphoid

follicle

T cell zone

(periarteriolar

lymphoid

sheath PALS)

Red pulp

B cell zone

(follicle)

Marginal

zone

Marginal

sinus

Follicular

arteriole

Trabecular

artery

Central

arteriole

A

Fig. 1.15 Morphology of the spleen. A, Schematic diagram

shows a splenic arteriole surrounded by the periarteriolar

lymphoid sheath (PALS) and attached follicles. The PALS

and lymphoid follicles together constitute the white pulp.

The marginal zone with its sinus is the indistinct boundary

between the white pulp and the red pulp. B, Light micrograph of a section of spleen shows an arteriole with the

PALS and a follicle with a prominent germinal center. These

are surrounded by the red pulp, which is rich in vascular

sinusoids.

CHAPTER 1 Introduction to the Immune System 17

• The cutaneous immune system and mucosal

immune system are specialized collections of lymphoid tissues and APCs located in and under the

epithelia of the skin and the gastrointestinal and

respiratory tracts, respectively. Although most

of the immune cells in these tissues are diffusely

scattered beneath the epithelial barriers, there

are discrete collections of lymphocytes and APCs

organized in a similar way as in lymph nodes. For

example, tonsils in the pharynx and Peyer patches

in the intestine are two anatomically defined

mucosal lymphoid tissues (Fig. 1.16). The immune

system of the skin consists of most of the cells of

innate and adaptive immunity, but without any

anatomically defined structures (Fig. 1.17). At any

time, at least a quarter of the body’s lymphocytes

are in the mucosal tissues and skin (reflecting the

large size of these tissues) (see Fig. 1.13), and many

Follicle

Dendritic

cell

Afferent

lymphatic

T cell Plasma cell Macrophage B cell

Peyer’s

patch

Lamina

propria

Mesentery

Mucosal

epithelium

M cell

Commensal

bacteria

Mucus

Crypt

Intraepithelial

lymphocytes

Intestinal

epithelial cell

Dendritic

cell

Lymphatic

drainage

Intestinal

lumen

Villus

Mesenteric

lymph node

IgA

Fig. 1.16 Mucosal immune system. Schematic diagram of the mucosal immune system uses the small

bowel as an example. Many commensal bacteria are present in the lumen. The mucus-secreting epithelium provides an innate barrier to microbial invasion (discussed in Chapter 2). Specialized epithelial

cells, such as M cells, promote the transport of antigens from the lumen into underlying tissues. Cells

in the lamina propria, including dendritic cells, T lymphocytes, and macrophages, provide innate and

adaptive immune defense against invading microbes; some of these cells are organized into specialized

structures, such as Peyer patches in the small intestine. Immunoglobulin A (IgA) is a type of antibody

abundantly produced in mucosal tissues that is transported into the lumen, where it binds and neutralizes

microbes (see Chapter 8).

18 CHAPTER 1 Introduction to the Immune System

of these are memory cells. Cutaneous and mucosal

lymphoid tissues are sites of immune responses to

antigens that breach epithelia. A remarkable property of the cutaneous and mucosal immune systems is that they are able to respond to pathogens

but do not react to the enormous numbers of usually harmless commensal microbes present at the

epithelial barriers. This is accomplished by several

mechanisms, including the action of regulatory T

cells and other cells that suppress rather than activate T lymphocytes.

Within the peripheral lymphoid organs, T lymphocytes and B lymphocytes are segregated into different anatomic compartments (Fig. 1.18). In lymph

nodes, the B cells are concentrated in discrete structures, called follicles, located around the periphery, or

cortex, of each node. If the B cells in a follicle have

recently responded to a protein antigen and received

signals from helper T cells, this follicle may contain

a central lightly staining region called a germinal

center. The germinal center has an important role in

the production of highly effective antibodies and is

described in Chapter 7. The T lymphocytes are concentrated outside but adjacent to the follicles, in the

paracortex. The follicles contain the FDCs described

earlier that are involved in the activation of B cells,

and the paracortex contains dendritic cells that present antigens to T lymphocytes. In the spleen, T lymphocytes are concentrated in periarteriolar lymphoid

sheaths surrounding small arterioles, and B cells reside

in the follicles.

The anatomic organization of peripheral lymphoid

organs is tightly regulated to allow immune responses

to develop after stimulation by antigens. B lymphocytes

are attracted to and retained in the follicles because of

the action of a class of cytokines called chemokines

(chemoattractant cytokines; chemokines and other

cytokines are discussed in more detail in later chapters).

FDCs in the follicles secrete a particular chemokine for

which naive B cells express a receptor, called CXCR5.

Postcapillary

venule

Keratinocytes

Commensal and

pathogenic microbes

Epidermis Dermis

Epidermal

Langerhans cell

Basal

keratinocytes

T lymphocyte

(CD4+)

Macrophage

Mast cell

Dermal

dendritic cell

Plasma cell

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