IDF Patient & Family Handbook for Primary Immunodeficiency ... - IDFA

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2 The Immune System And Primary Immunodeficiency Diseases Components of the Immune System The immune system is composed of a variety of different cell types and proteins. Each component performs a special task aimed at recognizing foreign material (antigens) and/or reacting against foreign material. For some components, recognition of the material as foreign to the body is their primary and only function. Other components function primarily to react against the foreign material. Still, other components function to both recognize and react against foreign materials. These foreign materials, or antigens, include microorganisms that cause infections (such as bacteria and viruses), pollen, and transplanted organs from other individuals. Since the functions of the immune system are so critical to survival, many of them can be performed by more than one component of the system. This redundancy acts as a back-up mechanism. Therefore, if one component of the whole system is missing or functioning poorly, another component can partially take over at least some of its functions. The major components of the immune system are: • B-lymphocytes • T-lymphocytes • NK cells • Phagocytes (Macrophages and Neutrophils) • Complement B-lymphocytes B-lymphocytes (sometimes called B-cells) are specialized cells of the immune system whose major function is to produce antibodies (also called immunoglobulins or gammaglobulins). B-lymphocytes develop from primitive cells (stem cells) in the bone marrow (see Figure 2). When mature, B-lymphocytes can be found in the bone marrow, lymph nodes, spleen, some areas of the intestine, and to a lesser extent, in the bloodstream. When B-lymphocytes are stimulated by a foreign material (antigens), they respond by maturing into another cell type called plasma cells. Plasma cells are the mature cells that actually produce the antibodies. Antibodies, the major product of plasma cells, find their way into the bloodstream, tissues, respiratory secretions, intestinal secretions, and even tears. Antibodies are highly specialized serum protein molecules. For every foreign antigen, there are antibody molecules specifically designed for that antigen. For example, like a lock and key, there are antibody molecules that physically fit the poliovirus, others that are aimed specifically at the bacteria that cause diphtheria and still others that match the measles virus. The variety of different antibody molecules is so extensive that B-lymphocytes have the ability to produce them against virtually all possible microorganisms in our environment. When antibody molecules recognize the microorganism as foreign, they physically attach to the microorganism and set off a complex chain of reactions involving other components of the immune system (see Figure 3) that eventually destroy the microorganism. The chemical names for antibody proteins are “immunoglobulins” or “gammaglobulins.” Antibodies vary from molecule to molecule with respect to which microorganisms they bind. They can also vary with respect to their specialized functions in the body (see Figure 4). This kind of variation in specialized function is determined by the antibody’s chemical structure, which in turn determines the class of the antibody (or immunoglobulin). There are four major classes of antibodies or immunoglobulins: • Immunoglobulin G (IgG) • Immunoglobulin A (IgA) • Immunoglobulin M (IgM) • Immunoglobulin E (IgE) Each immunoglobulin class has special chemical characteristics that provide it with specific advantages. For example, antibodies in the IgG fraction are formed in large quantities, last for over a month and travel from the blood stream to the tissues easily. The IgG class is the only class of immunoglobulins which crosses the placenta and passes immunity from the mother to the newborn. Antibodies of the IgA fraction are produced near mucus membranes and find their way into secretions such as tears, bile, saliva, and mucus, where they protect against infection in the respiratory tract and intestines. Antibodies of the IgM class are the first antibodies formed in response to infection. They are important in protection during the early days of an infection. Antibodies of the IgE class are responsible for allergic reactions.
The Immune System And Primary Immunodeficiency Diseases 3 CHAPTER 1; FIGURE 1 Major Organs of the Immune System A D B E C F G A. Thymus: The thymus is an organ located in the upper chest. Immature lymphocytes leave the bone marrow and find their way to the thymus where they are “educated” to become mature T-lymphocytes. B. Liver: The liver is the major organ responsible for synthesizing proteins of the complement system. In addition, it contains large numbers of phagocytic cells which ingest bacteria in the blood as it passes through the liver. C. Bone Marrow: The bone marrow is the location where all cells of the immune system begin their development from primitive stem cells. D. Tonsils: Tonsils are collections of lymphocytes in the throat. E. Lymph Nodes: Lymph nodes are collections of B-lymphocytes and T-lymphocytes throughout the body. Cells congregate in lymph nodes to communicate with each other. F. Spleen: The spleen is a collection of T-lymphocytes, B-lymphocytes and monocytes. It serves to filter the blood and provides a site for organisms and cells of the immune system to interact. G. Blood: Blood is the circulatory system that carries cells and proteins of the immune system from one part of the body to another.
Page 1 and 2: Patient & Family Handbook For Prima
Page 3 and 4: Patient & Family Handbook For Prima
Page 5 and 6: Patient and Family Handbook i Prefa
Page 7 and 8: Patient and Family Handbook iii Abo
Page 9: The Immune System and Primary Immun
Page 13 and 14: The Immune System And Primary Immun
Page 19 and 20: Common Variable Immune Deficiency c
Page 21 and 22: Common Variable Immune Deficiency 1
Page 23 and 24: X-Linked Agammaglobulinemia chapter
Page 25 and 26: X-Linked Agammaglobulinemia 17 Clin
Page 27 and 28: Selective IgA Deficiency chapter 4
Page 29 and 30: Selective IgA Deficiency 21 Clinica
Page 31 and 32: Severe Combined Immunodeficiency ch
Page 33 and 34: Severe Combined Immunodeficiency 25
Page 39 and 40: Chronic Granulomatous Disease 31 De
Page 41 and 42: Chronic Granulomatous Disease 33 Di
Page 43 and 44: Chronic Granulomatous Disease 35 Tr
Page 45 and 46: Wiskott-Aldrich Syndrome 37 Definit
Page 47 and 48: Wiskott-Aldrich Syndrome 39 Maligna
Page 49 and 50: Wiskott-Aldrich Syndrome 41 Treatme
Page 51 and 52: Hyper IgM Syndrome 43 Definition of
Page 53 and 54: Hyper IgM Syndrome 45 Treatment of
Page 55 and 56: DiGeorge Syndrome 47 Definition of
Page 57 and 58: DiGeorge Syndrome 49 Therapy for Di
Page 59 and 60: IgG Subclass Deficiency and Specifi
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IgG Subclass Deficiency and Specifi
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Ataxia Telangiectasia 55 Definition
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Ataxia Telangiectasia 57 General Tr
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Hyper IgE Syndrome 59 Definition of
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Hyper IgE Syndrome 61 Inheritance o
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Complement Deficiencies 63 Definiti
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Complement Deficiencies 65 Treatmen
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Other Important Primary Immunodefic
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Other Important Primary Immunodefic
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Inheritance chapter 15 Many disease
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Inheritance 73 Types of Inheritance
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Inheritance 75 Types of Inheritance
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Inheritance 77 Reproductive Options
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Laboratory Tests chapter 16 Laborat
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Laboratory Tests 81 Major Categorie
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Laboratory Tests 83 Summary Laborat
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General Care 85 General Health Meas
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General Care 87 Exercise Participat
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General Care 89 General Care During
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General Care 91 General Care During
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Specific Medical Therapy 93 Introdu
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Specific Medical Therapy 95 General
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Specific Medical Therapy 97 Hematop
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Specific Medical Therapy 99 Granulo
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Specific Medical Therapy 101 PEG-AD
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Specific Medical Therapy 103 Gene T
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Infants and Children with Primary I
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Adolescents with Primary Immunodefi
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Adults with Primary Immunodeficienc
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Health Insurance chapter 22 Having
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Health Insurance 127 Who Are the
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Health Insurance 133 LIFETIME MAXIM
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Glossary 135 Gamma globulins: The p
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Glossary 137 Sepsis: An infection o
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Reference 139 NIH Clinical Trials w
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Reference 141 International Patient
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Reference 143 Manufacturing Compani
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Reference 145 Books For Youth Balkw
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