2008 Immunology and Diseases

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Immunology and Diseases

Dr. Liao, Kuang-Wen

Dep. of Biological Science &Technology, NCTU


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Immuno-deficient Diseases

Hypersensitivity Diseases

Autoimmunity Tumor

Chronic Infectious Diseases


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Immuno-deficient Diseases

Inherited immunodeficiency diseases

Acquired immune deficiency syndromeoracquired immunodeficiency

syndrome (AIDS orAids)


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X-LINKED

AGAMMAGLOBULINEMIA (X

L-A):

This X-linked recessive disorder is due to a

developmental arrest in B-lymphocyte

differentiation. Males with X-LA have decreasednumbers of mature B-lymphocytes in blood and

severe panhypogammaglobulinemia.

Cell-mediated immune function is normal.

Individual infections may be no more severe

than in the general population but they may be

chronic or recurrent.


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SELECTIVE IGA DEFICIENCY

Selective IgA deficiency is the most prevalentprimary immunodeficiency disease, occurring inapproximately 1/500 to 1/1000 individuals in the

general population. Patients have serum IgA levels less than 5 mg/dl

with normal levels of other immunoglobulinclasses, normal serum antibody responses, and

normal cell mediated immunity. As many as 50% of patients with IgA deficiency

have chronic otitis, sinusitis or pneumonia.


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SEVERE COMBINED

IMMUNODEFICIENCYSevere Combine Immunodeficiency Disease(SCID) is a disorder characterized by a marked

deficiency of both B-lymphocyte and T-lymphocyte function.

One form of the autosomal recessive disease isdue to a deficiency ofAdenosine Deaminase.

Bone marrow transplantation is very effective butthe chances of a successful transplant depend inpart on the degree of infection and/or failure tothrive present at the time of transplantation.


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DIGEORGE ANOMALY

(SYNDROME):

Infants with this immunodeficiency have adevelopmental defect of their pharyngeal

pouches which affects embryogenesis oftheirparathyroids, thymus and hearts.

As a result, they may be born with

hypoparathyroidism and hypocalcemia,thymic aplasia and T- lymphocytedeficiency and congenital heart disease.


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Therapy?


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AIDS (Acquired immune deficiency

syndrome) Acquired immune deficiency syndrome oracquired immunodeficiency syndrome (AIDSorAids) is a collection of symptoms andinfections resulting from the specific damage to

the immune system caused by the humanimmunodeficiency virus (HIV).

HIV is transmitted through direct contact of a

mucous membrane or the bloodstream with abodily fluid containing HIV, such as blood,semen, vaginal fluid, preseminal fluid, andbreast milk.


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HIV is a retrovirus that primarily infects

vital components of the human immune

system such as CD4+ T cells (a subset ofT cells), macrophages and dendritic cells.

It directly and indirectly destroys CD4+ Tcells. When HIV kills CD4+ T cells so that

there are fewer than 200 CD4+ T cells per

microliter(L) ofblood, cellular immunity islost, leading to the condition known as

AIDS.


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Treatment

There is currently no vaccine or cure forHIVor AIDS.

The only known methods of prevention arebased on avoiding exposure to the virus.

Abacavir a nucleoside analog reversetranscriptase inhibitors (NARTIs or NRTIs)

The chemical structure of Abacavir


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Atazanavir a protease inhibitor

Current treatment for HIV infection consists ofhighly active antiretroviral therapy, or HAART.

This has been highly beneficial to many HIV-infected individuals since its introduction in 1996when the protease inhibitor-based HAART

initially became available.


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Current optimal HAART options consist of

combinations (or "cocktails") consisting of

at least three drugs belonging to at least

two types, or "classes," ofanti-retroviral

agents.

Typical regimens consist of two nucleosideanalogue reverse transcriptase inhibitors

(NARTIs or NRTIs) plus either a protease

inhibitoror a non-nucleoside reversetranscriptase inhibitor(NNRTI).


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How to cure AIDS


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Hypersensitivity Diseases

Type I Hypersensitivity Asthma

Type II Hypersensitivity Drug allergies

Type III Hypersensitivity

systemic lupus erythematosus

Type IV Hypersensitivity

Contact dermatitis (Th1)


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Type I Hypersensitivity

Immediate hypersensitivity is mediated by IgE.

The primary cellular component in this

hypersensitivity is the mast cell orbasophil. The mechanism of reaction involves preferential

production of IgE, in response to certain

antigens (allergens). IgE has very high affinity

for its receptor on mast cells and basophils.


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Type II Hypersensitivity

Type II hypersensitivity is also known ascytotoxic hypersensitivity and may affect avariety of organs and tissues.

The antigens are normally endogenous,although exogenous chemicals (haptens) whichcan attach to cell membranes can also lead totype II hypersensitivity.

Type II hypersensitivity is primarily mediated by

antibodies of the IgM or IgG classes andcomplement. Phagocytes and NK cells may alsoplay a role (ADCC).


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Type III Hypersensitivity

Type III hypersensitivity is also known as immunecomplex hypersensitivity.

The reaction may be general (e.g., serum sickness) ormay involve individual organs including skin (e.g.,systemic lupus erythematosus), kidneys (e.g., lupusnephritis), lungs (e.g., aspergillosis), blood vessels (e.g.,

polyarteritis), joints (e.g., rheumatoid arthritis) or otherorgans. This reaction may be the pathogenic mechanismof diseases caused by many microorganisms.


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Type IV Hypersensitivity

Type IV hypersensitivity is also known as cellmediated hypersensitivity.

Cytotoxic T cells (Tc) cause direct damagewhereas helper T (TH1) cells secrete cytokineswhich activate cytotoxic T cells and recruit andactivate monocytes and macrophages, which

cause the bulk of the damage. The delayedhypersensitivity lesions mainly containmonocytes and a few T cells.


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Autoimmunity

Definition

Autoimmune disorders are conditionscaused by an immune response against

the body's own tissues.


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Systemic Autoimmune Diseases

Localized Autoimmune Diseases


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2008 Immunology and Diseases

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