ABSTRACT
This book presents case histories to illustrate in a clinical context essential points about the mechanisms of immunity. It includes cases that illustrate both recently discovered genetic immunodeficiencies and some more familiar and common diseases with interesting immunology.
TABLE OF CONTENTS
chapter |6 pages
CASE 2 Chronic Granulomatous Disease
A specific failure of phagocytes to produce H and superoxide.
chapter |6 pages
CASE 5 Factor I Deficiency
The alternative pathway of complement activation is important in innate immunity.
chapter |6 pages
CASE 6 Deficiency of the C8 Complement Component
A loss of the lytic function of complement.
chapter |8 pages
CASE 7 Hereditary Periodic Fever Syndromes
Defects in innate immunity leading to an uncontrolled inflammatory response.
chapter |6 pages
CASE 8 Interleukin 1 Receptor-Associated Kinase 4 Deficiency
An inability of the innate immune system to detect the presence of pathogens.
chapter |6 pages
CASE 9 X-linked Hypohydrotic Ectodermal Dysplasia and Immunodeficiency
Immunodeficiency due to a defective component in an intracellular signaling pathway required for both innate and adaptive immunity.
chapter |4 pages
CASE 12 Activation-induced Cytidine Deaminase (AID) Deficiency
An intrinsic B-cell defect prevents immunoglobulin isotype switching.
chapter |6 pages
CASE 13 Common Variable Immunodeficiency
A failure to produce antibodies against particular antigens.
chapter |6 pages
Case 15 Adenosine Deaminase Deficiency 95
The purine degradation pathway and lymphocytes.
chapter |6 pages
CASE 16 Omenn Syndrome
A defect in V(D)J recombination results in severe immunodeficiency.
chapter |6 pages
Case 19 Multiple Myeloma 119
A malignancy of terminally differentiated B lymphocytes.
chapter |4 pages
Case 21 Interferon-g Receptor Deficiency 129
The destruction of intracellular microorganisms in macrophages.
chapter |8 pages
CASE 23 X-linked Lymphoproliferative Syndrome
A defect in the immune response to a virus.
chapter |6 pages
CASE 24 Autoimmune Lymphoproliferative Syndrome (ALPS)
Increased survival of lymphocytes as a result of a mutation in Fas.
chapter |6 pages
CASE 25 Immune Dysregulation, Polyendocrinopathy, Enteropathy X-linked Disease
A failure of peripheral tolerance due to defective
chapter |6 pages
CASE 26 Toxic Shock Syndrome
Superantigens cause excessive stimulation of T cells and macrophages.
chapter |4 pages
CASE 28 Mixed Essential Cryoglobulinemia
Chronic infection can lead to immune-complex disease.
chapter |6 pages
CASE 29 Rheumatic Fever
A streptococcal infection triggers an autoimmune disease of the heart.
chapter |6 pages
CASE 31 Acquired Immune Deficiency Syndrome (AIDS)
Infection can suppress adaptive immunity.
chapter |6 pages
CASE 32 Acute Systemic Anaphylaxis
A life-threatening immediate hypersensitivity reaction to peanuts.
chapter |8 pages
CASE 33 Allergic Asthma
Chronic allergic disease caused by an adaptive immune response to inhaled antigen.
chapter |6 pages
CASE 36 Celiac Disease
Chronic tissue damage in the gut caused by hypersensitivity to a common food antigen.
chapter |6 pages
CASE 37 Contact Sensitivity to Poison Ivy
A delayed hypersensitivity reaction to a hapten.
chapter |6 pages
CASE 38 Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy
Role of the thymus in the negative selection of T lymphocytes.
chapter |4 pages
CASE 42 Rheumatoid Arthritis
A common, severe inflammatory disease of unknown cause.
chapter |4 pages
CASE 44 Multiple Sclerosis
Autoimmune attack on the integrity of the central nervous system.
chapter |4 pages
CASE 45 Hemolytic Disease of the Newborn
The adaptive immune response distinguishes different individuals within a species.
chapter |6 pages
CASE 46 A Kidney Graft for Complications of Autoimmune Insulin-Dependent Diabetes Mellitus
Mechanisms of graft rejection and HLA associations
