CHAPTER  19  DISORDERS OF THE IMMUNE SYSTEM

 

HYPERSENSITIVITY (ALLERGY)

 

Allergies are a reaction to an antigen which is called an allergen. Allergens themselves are not harmful and do not provoke a reaction in everyone. Some individuals become sensitized to a particular allergen, which the immune systems of most of the population ignore. The resulting reaction causes tissue damage which can be anywhere from mildly annoying to miserable to fatal.

 

1. Type I (anaphylactic) reactions—these can be the most severe and dangerous allergic reactions.  They occur when IgE antibodies are formed against a particular allergen. These antibodies attach to mast cells and basophils. When the allergen enters the body and comes in contact with these antibodies, the chemical mediator histamine is rapidly released from granules inside the basophils and mast cells. These cells also produce and release other mediators such as leukotrienes and prostaglandins. The chemical mediators cause increased permeability of capillaries, increased secretion of mucus, smooth muscle contraction, etc.

     a. Systemic anaphylaxis—very large amounts of the chemical mediators are released and cause circulatory collapse and constriction of air passages which may lead to death. Epinephrine can counteract this.

     b. Local anaphylaxis—less severe, less release of mediators. Usually associated with ingested or inhaled antigens. Effects include hives, asthma, hay fever, gastrointestinal upsets, etc.

 

Repeated injections of tiny  amounts of the allergen may succeed in desensitization. IgG antibodies are formed, which block the antigen by binding to it before it can reach the IgEs.

 

2. Type II (cytotoxic reactions)—these are mediated by IgG or IgM antibodies and complement. This type of reaction is directed toward cells, which are lysed or damaged in other ways.

     a. Blood groups—“wrong” type blood cells given in a transfusion are an example of this type of reaction.

     b. Drug-induced cytotoxic reactions—drug molecules coat a blood cell or a platelet and the combination becomes antigenic, resulting in destruction of the cell or platelet. Complement proteins are involved.

                              Platelets attacked—thrombocytopenic purpura

                              White blood cells attacked—agranuloctosis

                               Red blood cells attacked—hemolytic anemia

 

3. Type III (immune complex) reactions---antigen-antibody complexes are deposited in organs and cause inflammatory damage. Complement becomes activated and this plus attraction and release of enzymes from neutrophils results in damage or destruction of the cells in the area.  Glomerulonephritis is an example.

 

4. Type IV (cell-mediated) reactions—also called delayed hypersensitivity reactions. T cells are involved in this type instead of antibodies. T cells become activated, release destructive cytokines, and attract macrophages to the area. Cytotoxic T cells may also appear. This results in damage to cells in the affected area. Contact dermatitis and poison ivy are examples. The skin test for tuberculosis is based on this type of reaction. Purified protein components of M. tuberculosis are injected. If the person has been previously exposed to the organism, sensitized T cells will react.

 

 

 

AUTOIMMUNE DISEASES

 

In spite of nature’s ways of preventing it, it is possible for one’s own immune system to lose the ability to distinguish self or to choose not to respond to self. The result is an attack on a tissue or tissues of the body.

 

1. Type I Autoimmunity--antibodies made in response to a virus find proteins on body cells that also react with the antibodies. Cells are destroyed.

 

2. Type II (Cytotoxic) autoimmune reactions involve production of antibodies which react to cell-surface antigens. Cells are not destroyed but other harmful effects occur. Myasthenia gravis and Grave’s disease are examples.

 

3. Type III ( Immune complex)  autoimmune reactions involve the deposition of immune complexes (antigen-antibody complexes) which result in tissue damage. Examples are lupus and rheumatoid arthritis.

 

4. Type IV (Cell-mediated) autoimmune reactions involve attack by cytolytic T cells. Examples are Hashimoto’s thyroiditis, multiple sclerosis, and Type I  diabetes mellitus.

 

 

 

DISEASES & REACTIONS RELATED TO THE HUMAN LEUKOCYTE ANTIGEN (HLA) COMPLEX

 

All nucleated human cells bear on the surface of their plasma membranes special protein complexes that are the thing the immune system uses to recognize self (or non-self). These proteins are capable of acting as antigens, but the immune system of the owner is supposed to recognize that these proteins and the cells they are attached to are self, and not respond to them. These proteins are known as MHC (major histocompatibility complex) proteins or HLA (human leukocyte antigens). These proteins are found on cells other than leukocytes, but leukocytes can be easily removed and classified, so that is the way this name arose.

 

These antigens are of major importance in tissue transplants. The ABO blood group and the HLA antigens must be matched as closely as possible between donor and recipient in an effort to prevent or minimize  the rejection of transplants. Certain HLA antigens may also be associated with increased susceptibility to certain diseases. HLA typing is the process used to identify the exact type of HLA antigens a person has.

 

To determine the suitability of a transplant between two persons, the ABO group must  first match. Then HLA typing further tests the chances of a successful transplant. Two main classes of HLAs are tested and matched, Class I HLAs and Class II HLAs. Class I HLAs are found on all cells in the body except RBC. Class II HLAs are found mostly on the surface of specialized immune cells. Ideally, both will be a close match, although they will not be identical unless an identical twin can act as the donor.

 

 

 

 

TYPES OF TRANSPLANTS AND THEIR CHANCES OF REJECTION

 

 

1. Privileged sites and tissues---this type of transplant normally is not rejected and does not require matching because it does not stimulate an immune response.

     a. Cornea

     b. Brain---one day we might be able to transplant nerves (but not yet)

     c. Valves from pigs’ hearts

     d. Pregnancy

 

2. Other transplants

     a. Autograft---some of your own tissue, such as skin, is transplanted to another site on the body

     b. Isograft---between  identical twins

     c. Allograft---between  two people who are not identical twins

     d. Xenograft---between different species--disasters now but some day we may grow pigs with human-like MHC proteins—transplanted tissue is called a xenotransplantation product.

 

3. Bone marrow transplants---this procedure is used in several circumstances:

     a. To give a person who cannot produce B and T cells that ability

     b. Leukemia patients

     c. Metastasis of other cancers to the bone marrow

The patient’s own bone marrow is destroyed and the transplant is given to replace it. Unfortunately, the new bone marrow sometimes produces immune cells that begin to attack the tissues of the recipient. This is called graft-versus-host disease.  Use of umbilical cord blood, which is rich in stem cells, may work better than transplanting bone marrow.

 

Persons who have received a transplant that might involve rejection must be treated to prevent that from happening. The major aim is to suppress cell-mediated immunity. The drugs used have side effects, so the benefits of the transplant must be weighed against the drawbacks.

 

 

 

IMMUNE DEFICIENCIES

 

1. Congenital deficiencies---person is born without some component of the immune system.

     a. DiGeorge syndrome---no thymus gland---no T cells---often fatal in infancy due to viral or fungal infections

     b. Severe combined immunodeficiency (boy in the bubble)---complete lack of the stem cells that produce both B and T cells

 

2. Acquired immune deficiencies---due to something that happens after birth

     a. AIDS is the best known

     b. Viruses, cancers, loss of the spleen, etc. are other causes

 

 

IMMUNE SYSTEM AND CANCER

 

Immunological surveillance--the immune system constantly looks for abnormal cells, including early cancers, and destroys them before they cause trouble. If this fails and a tumor develops, the cancer cells may be evading the immune system by:

  1. Not displaying antigens on their surface
  2. Releasing factors that interfere with cytotoxic T cells
  3. Causing immune cells to undergo apoptosis

 

Immunotherapy is treating cancer by means of the immune system. Possibilities:

  1. Monoclonal antibodies
  2. Immunotoxins
  3. Vaccines against certain cancers