Disorder Associated with the

Immune System
 What is Immune System?
The immune system is the body’s defense against
infectious organisms and other invaders.
What is Hypersensitivity?
 Hypersensitivity Reactions
• Response to antigens (allergens) leading to damage
• Types of hypersensitivity reactions
– Anaphylactic
– Cytotoxic
– Immune complex
– Delayed cell-mediated
 Type I (Anaphylactic) Reactions
• Occurs with in 2-30 mins.
• Antigen + IgE antibodies = Anaphylactic Reaction
• Mast cells and basophils undergo degranulation, which
releases mediators:
 Histamine - increase blood capillary
 Leukotrienes - increase blood capillary permeability
 Prostaglandins – increase smooth contraction and
mucus secretion
 Anaphylactic

• Anaphylaxis is a severe allergic reaction that

needs to be treated right away.
• If left untreated, it can be deadly.
Different and similarities of basophil and mast cell
Mast Cell
• Very similar in both appearance and function
• Develop from different hematopoietic lineages

Basophil
• Very similar in both appearance and function
• Develop from different hematopoietic lineages
• a white blood cell having a two-lobed nucleus
and basophilic granules in its cytoplasm
Systemic anaphylaxis (anaphylactic shock)
• Occurs inside the body system.
• It can be fatal within a few minutes
• Treatment: EPINEPHRINE
Systemic anaphylaxis
Allergen
• Insect venom
• Drugs blood circulation
• Food allergens
Localized anaphylaxis
• Target organ responds to direct contact with allergen
– Digestive tract contact results in vomiting,
cramping, diarrhea
– Skin sensitivity usually reddened inflamed area
resulting in itching
– Airway sensitivity results in sneezing and rhinitis
or wheezing and asthma
 Localized anaphylaxis

A micrograph of pollen grains A micrograph of a house mite on

fabric

Associated with the antigens that are ingested(food) or inhaled

This cause uticaria or oedema at the site of
exposure to the allergen, this may lead onto
a Full anaphylactic or anaphlactoid reaction
 Prevention of Anaphylactic Reaction
• Avoiding contact with
sensitizing antigens is the
most obvious way to prevent
allergic reaction
• Some individual experience
allergic reaction after eating
an assortment of food. In
such cases skin test might be
A skin test to identify allergens.
used to diagnosed
Type II Hypersensitivity
 Type II (Cytotoxic) Reactions
Type II hypersensitivity (cytotoxic) is mediated
by antibodies directed toward antigens
present on the surface of cells or other tissue
components.

Complement dependent reactions: Antibody is directed

against antigen on cells (such as circulating red blood cells)
or extracellular materials (basement membrane). The
resulting Ag-Ab complexes activate complement (via the
classic pathway), leading to cell lysis or extracellular tissue
damage.
a red blood cell has antigen fixed on its surface to which antibody
attaches. The attached antibody sets off the complement cascade,
which ends with the formation of the "membrane attack complex" of
C5-9 which causes lysis of the cell. Other complement components
may be generated, such as the opsonin C3b.
Diseases in this complement dependent category include:

•Transfusion reactions: incompatible RBC's or serum is transfused.

•Autoimmune hemolytic anemia: antibody is made against one's own RBC's.
•Erythroblastosis fetalis: maternal IgG crosses the placenta and attaches to
fetal RBC's.
•Goodpasture's syndrome: glomerular basement membrane antibody is
present.
Antibody-dependent cell-mediated cytotoxicity (ADCC): Low concentrations
of IgG or IgE (in the case of parasites) coat target cells. Inflammatory cells
such as NK (natural killer) cells, monocytes, and granulocytes then bind to
the immunoglobulin Fc receptors and lyse, but do not phagocytize, the target
cells.
Insert Table 19.2
 Placenta

Rh+ father. Rh– mother carrying In response to the If the woman becomes
her first Rh+ fetus. fetal Rh antigens, pregnant with another
Rh antigens from the mother will Rh+ fetus, her anti-Rh
the developing fetus produce anti-Rh antibodies will cross
can enter the antibodies. the placenta and
mother's blood damage fetal red blood
during delivery. cells.
Type III Hypersensitivity
Type IV (Cell-Mediated) Reactions

• Delayed-type hypersensitivities due to

T cells
• Cytokines attract macrophages and TC cells
– Initiate tissue damage
• Skin: Atopic dermatitis
• Lungs: Tuberculosis , hypersensitivity pneumonitis,
Granulomatosis with polyangiitis (formerly known as
Wegener’s granulomatosis)
• Pancreas: Type I diabetes mellitus, or known
previously as insulin-dependent diabetes mellitus.
• Thyroid: inflammation of the thyroid
• Other: transplant rejection, leprosy
Atopic dermatitis
Insert Fig 19.8

Allergic contact dermatitis

What Is the Delayed Rash?
A Delayed Rash, unnu
Autoimmune Diseases
 Autoimmune Diseases

• Immune system disorders cause abnormally low activity

or over activity of the immune system. In cases of
immune system over activity, the body attacks and
damages its own tissues (autoimmune diseases).
Immune deficiency diseases decrease the body's ability
to fight invaders, causing vulnerability to infections.
 Common Autoimmune Diseases
• Type 1 diabetes

• The pancreas produces the hormone insulin, which

helps regulate blood sugar levels. In type 1 diabetes,
the immune system attacks and destroys insulin-
producing cells in the pancreas.

• High blood sugar can damage blood vessels, as well

as organs like the heart, kidneys, eyes, and nerves.
 RHEUMATOID ARTHRITIS (AR) Systemic lupus erythematosus (lupus)

• Although doctors in the 1800s first

• In rheumatoid arthritis (RA), the
described lupus as a skin disease
immune system attacks the joints.
because of the rash it produces, it
This attack causes redness, warmth,
actually affects many organs, including
soreness, and stiffness in the joints.
the joints, kidneys, brain, and heart.
• Unlike osteoarthritis, which affects
people as they get older, RA can
• Joint pain, fatigue, and rashes are
start as early as your 30s .
among the most common symptoms.
• Treatment:METHOTREXATE
 Inflammatory bowel disease Addison’s disease

• Inflammatory bowel disease (IBD) is a

• It affects the adrenal glands, which
term used to describe conditions that
produce the hormones cortisol and
cause inflammation in the lining of the
aldosterone. Having too little of these
intestines. Each type of IBD affects a
hormones can affect the way the body
different part of the GI tract.
uses and stores carbohydrates and
• Crohn’s disease can inflame any part
sugar.
of the GI tract, from the mouth to the
anus.
• Symptoms include weakness, fatigue,
• Ulcerative colitisaffects only the lining
weight loss, and low blood sugar.
of the large intestine (colon) and
rectum.
 Graves’ disease
• It attacks the thyroid gland in the neck,
causing it to produce too much of its
hormones. Thyroid hormones control
the body’s energy usage, or
metabolism.
• One common symptom of this disease
is bulging eyes, called exophthalmos. It
affects up to 50 percent of people with
Graves’ disease
Sjögren’s syndrome
• the mucous membranes and
moisture-secreting glands of your
eyes and mouth are usually affected
first — resulting in decreased tears
and saliva.
• This condition attacks the joints, as
well as glands that provide lubrication
to the eyes and mouth. The hallmark
symptoms of Sjögren’s syndrome are
joint pain, dry eyes, and dry mouth.
 Hashimoto’s thyroiditis Vasculitis

• In Hashimoto’s thyroiditis, thyroid • Vasculitis happens when the immune

hormone production slows. system attacks blood vessels. The
Symptoms include weight gain, inflammation that results narrows the
sensitivity to cold, fatigue, hair loss, arteries and veins, allowing less blood to
and swelling of the thyroid (goiter). flow through them.
 Reaction related to the human
leukocyte anti agent(HLA) complex

HLA Reactions
• Histocompatibility antigens: self antigens on cell
surfaces
• Major histocompatibility complex (MHC): genes
encoding histocompatibility antigens
• Human leukocyte antigen (HLA) complex: MHC genes in
humans
 Diseases Related to Specific HLAs

Increased Risk of Occurrence

Disease
with Specific HLA

Multiple sclerosis 5 times

Rheumatic fever 4–5 times

Addison’s disease 4–10 times

Graves’ disease 10–12 times

Hodgkin’s disease 1.4–1.8 times

 Immune privilege

• Certain sites of the human body have immune privilege,

meaning they are able to tolerate the introduction of
antigens without eliciting an inflammatory immune
response.
Types of bone marrow transplant
• AUTOLOGOUS TRANSPLANTS
• Autologous transplants involve the use of a person’s own stem cells.

• ALLOGENEIC TRANSPLANTS
• Allogeneic transplants involve the use of cells from a donor.
• The success of an allogeneic transplant depends on how closely the donor
cells match your own.
 Immunosuppression

• Prevents an immune response to transplanted tissues

• Cyclosporine and tacrolimus suppress IL-2
• Mycophenolate mofetil inhibits T cell and B cell
reproduction
• Sirolimus blocks IL-2
• Basiliximab and daclizumab block IL-2
The interaction between a cytotoxic T lymphocyte (CTL) and a cancer cell.
Cancer cell Remains of cancer cell

CTL

CTL

The small CTL has The cancer cell has

already made a disintegrated.
perforation in the
cancer cell.
 Immunotherapy for Cancer

• Treatment of cancer using immunologic methods

• Tumor necrosis factor, IL-2, and interferons may kill
cancer cells
• Immunotoxins link poisons with a monoclonal
antibody directed at a tumor antigen
• Vaccines contain tumor-specific antigens
 Immunotherapy for Cancer
• Coley’s toxin (gram-negative bacteria)
stimulates TNF
• Vaccines used against:
– Marek’s disease
– Feline leukemia
– Human cervical cancer
– Liver cancer (hepatitis B virus)
– Cervical cancer (HPV vaccine)
• Monoclonal antibodies
– Herceptin
 Immunodeficiencies

• Congenital: due to defective or missing genes

• Acquired: develop during an individual’s life
– Due to drugs, cancers, and infections
Immunodeficiencies

Disease Cells Affected

AIDS TH (CD4+) cells
Selective IgA immunodeficiency B, T cells
Common variable B, T cells (decreased
hypogammaglobulinemia immunoglobulins)
Reticular dysgenesis B, T, and stem cells
Severe combined B, T, and stem cells
immunodeficiency
Thymic aplasia (DiGeorge T cells (defective
syndrome) thymus)
Wiskott-Aldrich syndrome B, T cells
X-linked infantile (Bruton’s) B cells (decreased
agammaglobulinemia immunoglobulins)
WHAT IS AIDS AND HIV?
 HIV
• Human Immunodeficiency
virus.
• A virus spread through certain
body fluids that attacks the
body’s immune system.
AIDS
• Acquired Immunodeficiency
virus.
• The final stage of HIV infection
when your immune system is
damage and to weak to fight
off ordinary infections.
 MAJOR DISEASES AND ILLNESSES
Meningitis

• inflammation of the meninges

caused by viral or bacterial
infection and marked by
intense headache and fever,
sensitivity to light, and
muscular rigidity, leading (in
severe cases) to convulsions,
delirium, and death.
 Encephalitis

• Encephalitis (en-sef-uh-LIE-tis)
is inflammation of the brain.
There are several causes, but
the most common is viral
infection. Encephalitis often
causes only mild flu-like signs
and symptoms — such as a
fever or headache — or no
symptoms at all.
 Tuberculosis
• is a disease caused by bacteria
called Mycobacterium
tuberculosis. The bacteria
usually attack the lungs, but
they can also damage other
parts of the body. TB spreads
through the air when a person
with TB of the lungs or throat
coughs, sneezes, or talks.
 Kaposi sarcoma (KS)
• is a cancer that causes
patches of abnormal tissue
to grow under the skin, in
the lining of the mouth,
nose, and throat, in lymph
nodes, or in other organs.
These patches, or lesions,
are usually red or purple.
They are made of cancer
cells, blood vessels, and
blood cells.
CAN WE CURE PEOPLE WITH HIV AND AIDS?
MEDICATION
Antiretroviral Drugs-doesn’t completely remove HIV from
the body but slows it down enough to enable your immune
system to fight infections.
CLASSES
• Entry/Fusion Inhibitors-distinguish the HIV infection
process by preventing the virus from attaching to the
cells.
• Ex. Enfuvirtide (Fuzeon), maraviroc (Selzentry)
• Reverse Transcriptase Inhibitors
Ex. Abacavir (Ziagen),Emtricitabine (Emtriva)
• Protease Inhibitors
Ex. ritonavir, saquinavir, and indinavir.
• Integrase Inhibitors
Ex. raltegravir , dolutegravir
HOW TO AVOID HIV INFECTION
• Know your HIV status and your patient’s status.
• Avoid vaginal and anal sex.
• Limited sex to one uninfected partner.
• Use condoms
• Avoid injectable drugs or shared needles.
• Avoid intoxication from drugs or alcohol.
Thank you!