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Chapter 16 Name the two major categories of immune dysfunction.

Hyposensitivities: Immune function is not completely developed, is suppressed, or destroyed. Primary immunodeficiency Secondary immunodeficiency - (AIDS) Hypersensitivities: Identify the four types of overreaction to antigens. Type I: Immediate hypersensitivity associated with exposure to specific antigens. IgE mediated; involves mast cells, basophils, and allergic mediators. Include anaphylaxis, allergies (atopy) such as hay fever, and asthma. Type II: Antibody mediated. IgG, IgM antibodies act upon cells with complement and cause cell lysis; includes some autoimmune diseases. Blood group incompatibility; pernicious anemia; myasthenia gravis. Type III: Immune complexmediated. Antibody-mediated inflammation; circulating IgG complexes deposited in basement membranes of target organs; includes some autoimmune diseases. Systemic lupus erythematosus; rheumatoid arthritis; serum sickness; rheumatic fever. Type IV: T-cell-mediated. Delayed hypersensitivity and cytotoxic reactions in tissues; includes some autoimmune diseases. Infection reactions; contact dermatitis; graft rejection. Define allergen and distinguish among inhalant, ingestant and contactant types. An allergen is an antigen that provokes an allergic response. Inhalants are airborne environmental allergens such as pollen, dust, dander (shed skin ), or fungal spores. Ingestants are allergens that enter by mouth, and often cause food allergies. Injectant allergies are an important adverse side effect of drugs or other substances used in diagnosing, treating, or preventing disease. A natural source of injectants is venom from stings by hymenopterans, a family of insects that includes honeybees and wasps. Contactants are allergens that enter through the skin. Describe the sequence of events after secondary exposures to allergens. After allergen is encountered again, the allergen attaches to IgE on mast cells and triggers degranulation and release of allergic mediators into the tissues and bloodstream. Cytokines then cause local and systemic reactions. The symptoms of allergy are not caused by the direct action of allergen on tissues but by the physiological effects of mast cell mediators on target organs. Explain why systemic anaphylaxis is so serious. Anaphylaxis can be accompanied by edema and vascular dilation, which leads to hypotension, tachycardia, circulatory failure, and shock. Anaphylactic persons have been known to die in 15 minutes from complete airway blockage. Briefly describe two methods for diagnosing allergies. Blood Testing: By measuring elevated blood levels of Tryptase, an enzyme released by mast cells that increase during an allergic response. Most widely used blood test is a radioallergosorbent (RAST) test, which measures levels of IgE to specific allergens. Skin Testing: An in vivo method to detect precise atopic or anaphylactic sensitivities. Unreliable for food allergies. Discuss the mechanism of action of allergy shots. A therapeutic way to prevent reactions between allergen, IgE, and mast cells. 16.08 List the major immune system components involved in Type II hypersensitivity.

16.09 Explain the basis for the ABO blood groups, and what type of antibody to the ABO antigens different individuals might have. 16.10 Identify which blood types are considered universal donors and universal recipients. 16.11 Explain under what circumstance the Rh factor can be problematic for newborn babies. 16.12 Specify how Type III hypersensitivity is similar to, and also differs from, Type II hypersensitivity. 16.13 Provide highlights about the Arthus reaction and serum sickness. 16.14 Describe the pathologenesis of contact dermatitis. 16.15 Provide the names for four different sources of graft material. 16.16 Name and describe at least three different theories of autoimmunity. 16.17 Describe the pathogenesis of at least three autoimmune diseases. 16.18 Distinguish between primary and secondary immunodeficiencies. 16.19 Explain what severe combined immunodeficiency is and discuss currently available therapeutic approaches. 16.20 Name three conditions that can cause secondary immunodeficiencies.

Allergy - The altered, usually exaggerated, immune response to an allergen. Also called hypersensitivity. Atopy - Allergic reaction classified as type me, with a strong familial relationship; caused by allergens such as pollen, insect venom, food, and dander; involves IgE antibodies. antibodies. Includes symptoms of hay fever, asthma, and skin rash. Histamine - A cytokine released when mast cells and basophils release their granules. An important mediator of allergy, its effects include smooth muscle Arthus reaction- An immune complex phenomenon that develops after repeat injection. This localized inflammation results from aggregates of antigen and antibody that bind, complement, and attract neutrophils. Autoantibodies- An anti-self antibody having an affinity for tissue antigens of the subject in which it is formed. Clonal Selection- A conceptional explanation for the development of lymphocyte specificity and variety during immune maturation. Cytokine- A chemical substance produced by white blood cells and tissue cells that regulates lymphocyte development, inflammation, and immunity. Systemic lupus erythematosus- A systemic autoimmune disease. All patients produce autoantibodies against a great variety of organs and tissues. Rheumatoid arthritisMyasthenia gravis-

SclerosisGraves' diseaseHashimoto's thyroiditisDiabetes mellitusLeukotrieneWheal Serotonin Leukotriene ProstaglandinBradykininGraftTransfusionCancer Allergen HypersensitivityAutoimmunityTuberculin reaction Contact dermatitis Graft versus host disease (GVHD)AutograftIsograftAllograft Xenograft Agammaglobulinemia Hypogammaglobulinemia DiGeorge syndromeSensitizing dose Provocative dose

AlloantigenImmunoglobulin E Mast cells Allergic rhinitis Asthma Rales Eczema-A general term for atopic dermatitis Atopic dermatitis Anaphylactic shock Desensitization Hyposensitization ABO blood types Rh factor Universal donor Universal recipient Erythroblastosis fetalis RhoGAM Immune complex reaction Serum sicknessSevere combined immunodeficiencies (SCID)Adenosine deaminase (ADA) deficiency Acquired immunodeficiency syndrome (AIDS)Cutaneous anaphylaxisSystemic anaphylaxisDegranulation-

16.1 The Immune Response: A Two-Sided Coin Immunopathology is the study of diseases associated with excesses and deficiencies of the immune response. Such diseases include allergies, autoimmunity, grafts, transfusions, immunodeficiency disease, and cancer. There are four categories of hypersensitivity reactions: Type I (allergy and anaphylaxis), type II (IgG and IgM tissue destruction), type III (immune complex reactions), and type IV (delayed hypersensitivity reactions). 16.2 Type I Allergic Reactions: Atopy and Anaphylaxis An allergy or hypersensitivity is an exaggerated immune response that injures or inflames tissues. Antigens that trigger hypersensitivity reactions are allergens. They can be either exogenous (originate outside the host) or endogenous (involve the hosts own tissue). Type I hypersensitivity reactions result from excessive IgE production in response to an exogenous antigen. The two kinds of type I hypersensitivities are atopy, a chronic, local allergy, and anaphylaxis, a systemic, potentially fatal allergic response. The predisposition to type I hypersensitivities is inherited, but age, geographic locale, and infection also influence allergic response. Type I allergens include inhalants, ingestants, injectants, and contactants. The portals of entry for type I antigens are the skin, respiratory tract, gastrointestinal tract, and genitourinary tract. Type I hypersensitivities are set up by a sensitizing dose of allergen and expressed when a second provocative dose triggers the allergic response. The time interval between the two can be many years. The primary participants in type I hypersensitivities are IgE, basophils, mast cells, and agents of the inflammatory response. Allergies are diagnosed by a variety of in vitro and in vivo tests that assay specifi c cells, IgE, and local reactions. Allergies are treated by medications that interrupt the allergic response at certain points. Allergic reactions can often be prevented by desensitization therapy. 16.3 Type II Hypersensitivities: Reactions That Lyse Foreign Cells Type II hypersensitivity reactions occur when preformed antibodies react with foreign cell-bound antigens. The most common type II reactions occur when transfused blood is mismatched to the recipients ABO type. IgG or IgM antibodies attach to the foreign cells, resulting in complement fixation. The resultant formation of membrane attack complexes lyses the donor cells. Complement, IgG, and IgM antibodies are the primary mediators of type II hypersensitivities. The concepts of universal donor (type O) and universal recipient (type AB) apply only under emergency circumstances. Cross-matching donor and recipient blood is necessary to determine which transfusions are safe to perform. Type II hypersensitivities can also occur when Rhmothers are sensitized to Rh+ RBCs of their unborn babies and the mothers anti-Rh antibodies cross the placenta, causing hemolysis of the newborns RBCs. This is called hemolytic disease of the newborn, or erythroblastosis fetalis. 16.4 Type III Hypersensitivities: Immune Complex Reactions Type III hypersensitivity reactions occur when large quantities of antigen react with host antibody to form small, soluble immune complexes that settle in tissue cell membranes, causing chronic destructive infl ammation. The reactions appear hours or days after the antigen challenge. The mediators of type III hypersensitivity reactions include soluble IgA, IgG, or IgM, and agents of the infl ammatory response. Two kinds of type III hypersensitivities are localized (Arthus) reactions and systemic (serum sickness).

16.5 Type IV Hypersensitivities: Cell-Mediated (Delayed) Reactions Type IV hypersensitivity reactions occur when cytotoxic T cells attack either self tissue or transplanted foreign cells. Type IV reactions are also termed delayed hypersensitivity reactions because they occur hours to days after the antigenic challenge. Type IV hypersensitivity reactions are mediated by T lymphocytes and are carried out against foreign cells that show both a foreign MHC and a nonself receptor site. Examples of type IV reactions include the tuberculin reaction, contact dermatitis, and mismatched organ transplants (host rejection and GVHD reactions). The four classes of transplants or grafts are determined by the degree of MHC similarity between graft and host. From most to least similar, these are autografts, isografts, allografts, and xenografts. Graft rejection can be minimized by tissue matching procedures, immunosuppressive drugs, and use of tissues that do not provoke a type IV response. 16.6 An Inappropriate Response Against Self: Autoimmunity Autoimmune hypersensitivity reactions occur when autoantibodies or host T cells mount an abnormal attack against self antigens. Susceptibility to autoimmune disease appears to be infl uenced by gender and by genes in the MHC complex. Autoimmune disease may be an excessive response of a normal immune function, the appearance of sequestered antigens, forbidden clones of lymphocytes that react to self antigens, or the result of alterations in the immune response caused by infectious agents, particularly viruses. Examples of autoimmune diseases include systemic lupus erythematosus, rheumatoid arthritis, diabetes mellitus, myasthenia gravis, and multiple sclerosis. 16.7 Immunodeficiency Diseases: Hyposensitivity of the Immune System Immunodeficiency diseases occur when the immune response is reduced or absent. Primary immune diseases are genetically induced defi -ciencies of B cells, T cells, the thymus gland, or combinations of these. Secondary immune diseases are caused by infection, organic disease, chemotherapy, or radiation. The best-known infection-induced immunodeficiency is AIDS.

1. Pollen is which type of allergen? a. contactant b. ingestant c. injectant d. inhalant

2. B cells are responsible for which allergies? a. asthma b. anaphylaxis c. tuberculin reactions d. both a and b

3. The contact with allergen that results in symptoms is called the a. sensitizing dose c. provocative dose. b. degranulation dose. d. desensitizing dose.

4. The direct, immediate cause of allergic symptoms is the action of a. the allergen directly on smooth muscle. b. the allergen on B lymphocytes. c. allergic mediators released from mast cells and basophils. d. IgE on smooth muscle. 5. Theoretically, type _____ blood can be donated to all persons because it lacks _____. a. AB, antibodies c. AB, antigens b. O, antigens d. O, antibodies 6. An example of a type III immune complex disease is a. serum sickness. b. contact dermatitis. c. graft rejection. d. atopy

7. Type II hypersensitivities are due to a. IgE reacting with mast cells. b. activation of cytotoxic T cells. c. IgG-allergen complexes that clog epithelial tissues. d. complement-induced lysis of cells in the presence of antibodies. 8. Production of autoantibodies may be due to a. emergence of forbidden clones of B cells. b. production of antibodies against sequestered tissues. c. infection-induced change in receptors. d. all of these are possible. 9. Rheumatoid arthritis is an ____ that affects the ____. a. immunodeficiency disease, muscles b. autoimmune disease, nerves c. allergy, cartilage d. autoimmune disease, joints 10. Which disease would be most similar to AIDS in its pathology? a. X-linked agammaglobulinemia b. SCID c. ADA deficiency d. DiGeorge syndrome 11. T cells are associated with type IV allergies. TRUE 12. A positive tuberculin skin test is an example of antibody mediated inflammation. FALSE: A positive TB skin test is an example of delayed hypersensitivity. 13. Contact dermatitis can be caused by proteins found in foods. FALSE: Contact dermatitis can be caused by chemicals absorbed through the skin. 14. Antibody-mediated degranulation of mast cells is involved in anaphylaxis. TRUE 15. Rejection of transplanted tissue is dependent on MHC/HLA markers. TRUE