The document discusses disorders of the immune system. It describes the functions of a normal immune system and what can go wrong, including immunodeficiencies where the immune system is weak or overactive. It covers specific immune disorders like allergies, autoimmune diseases, and transplant rejection. It also discusses cancer immunology and HIV/AIDS.

1. DISORDERS OF THE
IMMUNE SYSTEM

2. FUNCTIONS OF THE IMMUNE SYSTEM
to protect against disease or other potentially damaging
foreign bodies.
identifies a variety of threats, including viruses, bacteria
and parasites, and distinguishes them from the body's own
healthy tissue

3. WHAT CAN GO WRONG WITH THE IMMUNE
SYSTEM?
When your immune system doesn't work the way it should, it is
called an immune system or immunodeficiency disorder.

4. SOMEONE MAY BE…..
Be born with a weak immune system. This is called
primary immune deficiency.
Get a disease that weakens your immune system. This is
called acquired immune deficiency.
Have an immune system that is too active.
Have an immune system that turns against you.
Conditions called autoimmune disease occur.

5. AN OVERACTIVE IMMUNE
SYSTEM/HYPERSENSITIVITY
If you are born with certain genes, your immune system may
react to substances in the environment that are normally
harmless.

6. FOUR PRINCIPAL TYPES OF HYPERSENSITVITY
Type I (Anaphylactic)
 < 30 min symptoms occur.
 IgE binds to mast cells or basophils: causes
degranulation of mast cell or basophil and release of
reactive substances such as histamine
 Examples:
 Anaphylactic shock from drug injections and insect
venom ,hay fever, asthma

7. Anaphylactic responses can be…
systemic reactions
shock and breathing difficulties
localized reactions
hay fever, asthma, and hives (slightly raised, often itchy and
reddened areas of the skin).

8. Asthma
The response in your lungs can cause
coughing, wheezing, and trouble
breathing. Asthma can be triggered
by a common allergen like dust or
pollen or by an irritant like tobacco smoke.

10. Allergic rhinitis
Sneezing, a runny nose, sniffling, and
swelling of your nasal passages from
indoor allergens like dust and pets or
outdoor allergens like pollens or molds.

11. Eczema
An allergen causes an itchy rash known as atopic dermatitis

12. SKIN TESTING

13. TYPE II (CYTOTOXIC)
 Clinical symptoms occur 5-12 hours
 Antigen causes formation of IgM and IgG antibodies that
bind to target cell , when combined with action of
complement, destroys target cell
 -Examples: Transfusion reactions, Rh Incompatibility, HDNB

14. Blood Transfusions and Rh Incompatibility
If blood from an Rh + donor is given to an Rh - recipient, the
donor's RBCs stimulate the production of anti-Rh antibodies
in the recipient. If the recipient then receives Rh + RBCs in a
subsequent transfusion, a rapid, serious hemolytic reaction
will develop.

15. HEMOLYTIC DISEASE OF THE NEWBORN
Also called erythroblastosis fetalis
incompatibility between the blood types of the mother and
the baby.
HEMOLYTIC- breaking down of red blood cells.
Erythroblastosis-making of immature red blood cells.

16. HDNB is usually prevented today by passive immunization of
the Rh - mother at the time of delivery of any Rh + infant with
anti-Rh antibodies(RhoGAM).

17. DRUG-INDUCED CYTOTOXIC REACTIONS
Thrombocytopenic purpura
Blood platelets (thrombocytes) are destroyed by drug-
induced cytotoxic reactions.

18. Drugs may bind similarly to white or red blood cells, causing
local hemorrhaging and yielding symptoms described as
"blueberry muffin" skin mottling.

19. BLUEBERRY MUFFIN (SKIN MOTTLING)

20. Agranulocytosis
-immune-caused destruction of agranulocytic white cells and
it affects the body's phagocytic defenses.
Hemolytic anemia
-when RBCs are destroyed

21. TYPE III (IMMUNE COMPLEX)
 clinical symptoms occur 3-8 hours
 Antibodies and antigens form complexes that cause
damaging inflammation
 Examples: Arthus reactions, serum sickness

22. Arthus Reaction- an acute response to a second injection of
vaccines (boosters)or drugs at the same site as the first
injection.
Serum sickness-condition appeared in soldiers after repeated
injection of horse serum to treat tetanus. It can also be
caused by injection of animal hormones and drugs.

23.  Glomerulonephritis is an immune complex condition,
usually resulting from an infection, that causes
inflammatory damage to the kidney glomeruli.

24. TYPE IV (DELAYED CELL- MEDIATED, OR DELAYED
HYPERSENSITIVITY)
 clinical signs occur 24-48 hours
 Antigens activate T cells that kill target cell
 Examples: Rejection of transplanted tissues, contact
dermatitis ,tuberculosis

25. ALLERGIC CONTACT DERMATITIS

26. AUTOIMMUNE DISEASE
In autoimmune diseases, the body attacks normal, healthy
tissues. The cause is unknown. It is probably a combination
of a person's genes and something in the environment that
triggers those genes.

27. CYTOTOXIC AUTOIMMUNE REACTIONS
Graves' disease
The immune system produces antibodies that stimulate the
thyroid gland to release excess amounts of thyroid
hormone into the blood (hyperthyroidism).
Symptoms: bulging eyes weight loss, nervousness, irritability,
rapid heart rate, weakness, and brittle hair.
Treatment:Destruction or removal of the thyroid gland

28. GRAVES DISEASE

29. Myasthenia gravis
Antibodies bind to nerves and make them unable to
stimulate muscles properly.
Weakness that gets worse with activity is the main symptom
of myasthenia gravis.
Mestinon (pyridostigmine) is the main medicine used to treat
myasthenia gravis.

30. MYASTHENIA GRAVIS

31. IMMUNE COMPLEX AUTOIMMUNE REACTIONS
Systemic lupus erythematosus (lupus)
People with lupus develop autoimmune antibodies that
can attach to tissues throughout the body.
 The joints, lungs, blood cells, nerves, and kidneys are
commonly affected in lupus.
 Treatment often requires daily oral prednisone.

32. SYSTEMIC LUPUS ERYTHEMATOSUS

33. Rheumatoid arthritis
The immune system produces antibodies that attach to the
linings of joints.
Immune system cells then attack the joints, causing
inflammation, swelling, and pain
Treatments for rheumatoid arthritis can include various oral
or injectable medications that reduce immune system over
activity.

34. RHEUMATOID ARTHRITIS

35. CELL-MEDIATED AUTOIMMUNE REACTIONS
Type 1 diabetes
In this type of diabetes, the immune system attacks the
cells in the pancreas that make insulin.

36. Psoriasis
In psoriasis, overactive immune system blood cells called T-
cells collect in the skin. The immune system activity
stimulates skin cells to reproduce rapidly, producing silvery,
scaly plaques on the skin.

37. PSORIASIS

38. REACTIONS RELATED TO THE HUMAN
LEUKOCYTE ANTIGEN (HLA) COMPLEX
Human Leukocyte Antigen (HLA)
- Is the locus of genes that encode for proteins on the surface
of cells that are responsible for regulation of the immune
system in humans.

39. Certain HLAs are related to an increased susceptibility
to specific diseases.

40. HLA typing
-used to identify and compare HLAs.
Another important medical application of HLA typing is in
transplant surgery, in which the donor and the recipient must be
matched by tissue typing
Serological tissue typing
-the laboratory uses standardized antisera or monoclonal
antibodies that are specific for particular HLAs.

41. Tissue typing
-Lymphocytes from the person being tested are incubated
with laboratory test stocks of anti -HLA antibodies specific for a
particular HLA. If the antibodies react with the antigens on a
lymphocyte then complement damages the lymphocyte and
they can enter the cell.

42. Polymerase Chain Reaction
-new technique for analysing HLA is the use of the polymerase
chain reaction, to amplify the cell's DNA

43. DISEASES RELATED TO SPECIFIC HUMAN LEUKOCYTE
ANTIGENS (HLAS)
Disease Increased Risk of
Occurrence
with Specific
HLA"
Description
Inflammatory Diseases
Multiple sclerosis
Rheumatic fever
5 times
4- 5 times
Progressive inflammatory disease affecting
nervous system
Cross-reaction with antibodies against
streptococcal antigen
Endocrine Diseases
Addison's disease
Graves' disease
4- 10 times
10- 12 times
Deficiency in production of hormones by adrenal
gland
Antibodies attached to certain receptors in the
thyroid gland
cause it to enlarge and produce excessive
hormones
Malignant Disease
Hodgkin's disease 1.4- 1.8 times Cancer of lymph nodes

44. RHEUMATIC FEVER

45. ADISSON’S DISEASE

46. HODGKINS’S DISEASE

47. REACTIONS TO TRANSPLANTATION
Transplants recognized as non self are rejected- attacked by T
cells that directly lyse the grafted cells.

48. TYPES OF TRANSPLANTS
bone marrow
 lungs
 heart
 liver
 cornea

49. PRIVILEGED SITES AND PRIVILEGED TISSUE
Immunologically privileged site.
A part of the body wherein antibodies usually do not circulate,
meaning transplants do not stimulate immune response.
Ex: Corneal transplant

50. Privileged tissue
An example is replacing a person's damaged heart
valve with a valve from a pig's heart.

51. STEM CELLS
Embryonic Stem Cells (ESCs)
These cells can be isolated from the very earliest stage of
an embryo, usually from discarded embryos created for
attempts at in vitro fertilization.
ESCs are capable of generating many different types of
tissue cells and cell lines such as muscle, nerve, or blood
cells

52. GRAFTS
AUTOGRAFT
-when one's own tissue is grafted to another part of
the body.

53. ISOGRAFT
Identical twins have the same genetic makeup;
therefore, skin or organs such as kidneys may be
transplanted between them without provoking an
immune response.

54. ALLOGRAFTS
Grafts between people who are not identical twins.

55. XENOTRANSPLANTATION
Tissues or organs that have been transplanted from animals.

56. Hyperacute rejection occurs in human-to-human
transplants only when antibodies have been
preformed because of previous transfusions,
transplantations, or pregnancies.

57. Bone Marrow Transplants
The recipients are usually individuals who lack the
capacity to produce B cells and T cells vital for
immunity or who are suffering from leukemia.

58. Graft-Versus-Host(GVH) Disease
the transplanted bone marrow contains immunocompetent
cells that mount primarily a cell-mediated immune response
against the tissue into which they have been transplanted .

59. IMMUNOSUPRESSION
-to suppress cell-mediated immunity, the most important
factor in transplant rejection.
Cyclosporine and Tacrolimus (FKS06)
-suppresses the secretion of interleukin-2 (IL-2),
disrupting cell -mediated immunity by cytotoxic T
cells.

60. Cyclosporine and Tacrolimus (FKS06)
 suppresses the secretion of interleukin-2 (IL-2), disrupting
cell -mediated immunity by cytotoxic T cells.
 both has much effect on antibody production by the
humoral immune system.

61. Sirolimus(Rapamune)
-are among those that inhibit both cell-mediated and
humoral immunity.

62. Mycophenolate Mofetil
-inhibit the proliferation of T cells and B cells.
Some biological agents such as the chimeric
monoclonal antibodies basiliximab and daclizumab
also block IL-2 and are useful immunosuppressives.

63. CANCER

64. CANCER
 New growth of abnormal cells.
 Benign tumor -self contained mass within an organ that
does not spread into adjacent tissues.
 Malignant tumor -uncontrolled growth of abnormal cells
within normal tissues.

65. IMMUNOTHERAPY FOR CANCER
Herceptin
consists of monoclonal antibodies against a breast
cancer growth factor.
Immunotoxins
are chemical poisons linked to a monoclonal antibody ;
the antibody selectively locates the cancer cell for
release of the poison.

66. IMMUNOTHERAPY FOR CANCER
1.Non specific immune stimulation
2.Adoptive cell transfer
3.Immune checkpoint blockage
4.Vaccination strategies

67. IMMUNODEFICIENCIES
The absence of a sufficient immune which can be
either congenital or acquired.

68. Congenital Immunodeficiency
defects in, or the absence of a number of inherited
genes.

69. Severe combined immunodeficiency
(SCID). This is an example of an immune
deficiency that is present at birth. Children with
SCID are missing important white blood cells.

70. BUBBLE BABY DISEASE

71. ACQUIRED IMMUNODEFICIENCY
a variety of drugs, cancers, or infectious agents

72. SELECTIVE IGA IMMUNODEFICIENCY
B, T cells
Affects about 1 in 700, causing frequent mucosal
infections
specific cause uncertain

73. MUCOSAL INFECTIONS

74. COMMON VARIABLE
HYPOGAMMAGLOBULINEMIA
B, T cells (decreased immunoglobulins)
Frequent viral and bacterial infections second most
common immune deficiency, affecting about 1 in
70,000
inherited

75. HYPOGAMMAGLOBULINEMIA

76. RETICULAR DYSGENESIS
B, T. and stem cells (a combined
immunodeficiency: deficiencies in Band T cells and
neutrophils)
Usually fatal in early infancy:
inherited

77. RETICULAR DYSGENESIS

78. SEVERE COMBINED
IMMUNODEFICIENCY
B. T. and stem cells(deficiency of both Band T cells)
Affects about 1 in 100,000: allows severe infections:
Inherited

79. THYMIC APLASIA
(DIGEORGE SYNDROME)
T cells (defective thymus causes deficiency of T cells)
Absence of cell-mediated immunity; usually fatal in
infancy
from Pneumocyslis pneumonia or viral or fungal
infections: due to failure of the thymus to develop in
embryo

80. DIGEORGE SYNDROME

81. WISKOTT-ALDRICH SYNDROME
B, T cells (few platelets in blood, abnormal T cel ls)
Frequent infections by viruses, fungi, protozoa:
eczema,
defective blood clotting; usually causes death in
childhood:
inherited on X chromosome

82. WISKOTT-ALDRICH SYNDROME

83. X-LINKED INFANTILE (BRUTON'S)
AGAMMAGLOBULINEMIA
B cells (decreased immunoglobulins)
Frequent extracellular bacterial infections; affects
about 1 in 200,000: the first immunodeficiency
disorder recognized (1952):
inherited on X chromosome

84. HIV (HUMAN IMMUNODEFICIENCY VIRUS)
INFECTION
HIV (human immunodeficiency virus) is a virus that
attacks the immune system.
HIV infects and destroys certain white blood cells
called CD4+ cells.

85. AIDS (ACQUIRED IMMUNODEFICIENCY
SYNDROME)
 The last stage of HIV infection .
 People with AIDS have a low number of CD4+ cells and get
infections or cancers that rarely occur in healthy people.
These can be deadly.

86.  But having HIV doesn't mean you have AIDS. Even without
treatment, it takes a long time for HIV to progress to AIDS—
usually 10 to 12 years.
 With treatment, many people with HIV are able to live long
and active lives.

87. AIDS

88. TWO TYPES OF HIV
HIV-1-which causes almost all the cases of AIDS
worldwide
HIV-2-which causes an AIDS-like illness. HIV-2 infection
is uncommon in North America.

89. SIGNS AND SYMPTOMS
 Symptoms may appear from a few days to several weeks
after a person is first infected. The early symptoms usually go
away within 2 to 3 weeks.

90. After the early symptoms go away, an infected person may not
have symptoms again for many years. After a certain point,
symptoms reappear and then remain. These symptoms usually
include:
 Swollen lymph nodes.
 Extreme tiredness.
 Weight loss.
 Fever.
 Night sweats.

91. RISK FACTORS
 contact with infected blood, semen, or vaginal fluids.
 unprotected sex with someone who has HIV.
 sharing drug needles with someone who is infected with
HIV.
 during pregnancy, birth, or breast-feeding.
 casual contact like kissing or sharing drinking glasses with
an infected person.

92. DIAGNOSTIC EXAMINATION
 A doctor may suspect HIV if symptoms last and no other
cause can be found.
 If you have been exposed to HIV, your immune system will
make antibodies to try to destroy the virus. Doctors use tests
to find these antibodies in urine, saliva or blood.
 Viral load which shows the amount of virus in your blood.
 CD4+ cell count, which shows how well your immune system
is working.

93. Most doctors use two blood tests, called the ELISA and the
Western blot. If the ELISA is positive (meaning that HIV
antibodies are found), a Western blot or other test will be
done to be sure.
 It may take as long as 6 months for HIV antibodies to show
up in your blood. If you think you have been exposed to HIV
but you test negative for it:
 Get tested again. Tests at 6, 12, and 24 weeks can be done
to be sure you are not infected.

94.  You can get HIV testing in most doctors' offices, public
health clinics, hospitals, and Planned Parenthood clinics.
 You can also buy a home HIV test kit in a drugstore or by
mail order. Make sure it's one that is approved by the
Food and Drug Administration (FDA). If a home test is
positive, see a doctor to have the result confirmed and
to find out what to do next.

95. TREATMENT
 The standard treatment for HIV is a combination of
medicines called antiretroviral therapy, or ART.
Antiretroviral medicines slow the rate at which the virus
multiplies.
 Taking these medicines can reduce the amount of virus in
your body and help you stay healthy.
 Medical experts recommend that people begin treatment
for HIV as soon as they know that they are infected.

96.  After you start treatment, it's important to take your
medicines exactly as directed by your doctor. When
treatment doesn't work, it is often because HIV has
become resistant to the medicine. This can happen if you
don't take your medicines correctly.

97. PREVENTION
 HIV is often spread by people who don't know they have it. So it's
always important to protect yourself and others by taking these
steps:
 Practice safer sex. Use a condom every time you have sex
(including oral sex) until you are sure that you and your partner
aren't infected with HIV or other sexually transmitted infection (STI).
 Don't have more than one sex partner at a time. The safest sex is
with one partner who has sex only with you.
 Talk to your partner before you have sex the first time. Find out if he
or she is at risk for HIV. Get tested together. Getting tested again at
6, 12, and 24 weeks after the first test can be done to be sure
neither of you is infected. Use condoms in the meantime.

98. Don't drink a lot of alcohol or use illegal drugs before
sex. You might let down your guard and not practice safer
sex.
Don't share personal items, such as toothbrushes or razors.
Never share needles or syringes with anyone.
You also can take antiretroviral medicine to help protect
yourself from HIV infection. But to keep your risk low, you still
need to practice safer sex even while you are taking the
medicine.

99. THE STAGES OF HIV INFECTION
Phase I
The number of viral RNA molecules per milliliter of
blood plasma may reach more than 10 million in the first
week. Billions of CD4 + T cells may be infected within a
couple of weeks.

100.  Immune responses and fewer uninfected cells to target
 deplete viral numbers in blood plasma sharply within a few
 weeks.
 The infection may be asymptomatic or cause
lymphadenopathy (swollen lymph nodes).

101. Phase 2
 The numbers of CD4 + T cells decline steadily.
 HIV replication continues but at a relatively low level,
probably controlled by CDS+ T cells mainly in lymphatic
tissue.
 Other early HIV symptoms include slight fever, headaches,
fatigue, and muscle aches. These symptoms may last for only
a few weeks. Then there are usually no HIV symptoms for
many years. That is why it can be hard to know if you have
HIV.

102. Phase 3
 -Clinical AIDS emerges, usually within 10 years of
infection.
 CD4 + T cell counts are below 350 cells/f.ll (200 cells/f.ll
defines AIDS).
 Important AIDS indicator conditions appear,such as C. albicaus
infections of bronchi, trachea, or lungs;
 cytomegalovirus eye infections; tuberculosis; Puel/moeys/is
pneumonia; toxoplasmosis of the brain; and Kaposi's sarcoma.