Disorders of the immune system

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Questions and Answers

What is hypersensitivity?

Antigenic response beyond that which is considered normal

What is an allergy?

Condition of altered reactivity or exaggerate immune response manifested by inflammation

Type 1 hypersensitivity?

Only one that’s allergic. IgE antibodies binds to mast cell and basophils. First time its binds it asymptomatic, antigen second time causes an immune response. Releases chemical mediators and leads to anaphylaxis. IgE usually binds to mast cell or basophil. The granule contains the chemical mediator. B cell sees it forgiven and make ok as a cell which makes IgE, which binds to mast ore basophil. Second time degranulization occurs releasing chemical mediators.

Type 1 hypersensitivity sequence?

<p>B cell see allergen and recognize it as foreign, B cell activated &amp; divie to get plasma cell. Plasma makes IgE only. IgE is priming it to be an allergic reaction and it binds to mast cell or basophil. It comes in contact with allergen, second time. Allergen binds to IgE, causing degranulization or rupturing of granules and releases chemical mediators. symptoms causes by the release of chemical mediators. This leads mainly to anaphylaxis. Occurs immediately.</p> Signup and view all the answers

RAST test

<p>By blood that does allergy testing. Test blood and not patient. Not as sensitive as skin test but safer for patient.</p> Signup and view all the answers

What are the conditions of hypersensitivity 1?

<p>Atopy: chronic local allergy Anaphylaxis; systemic. Explosive reaction. Airway obstruction, circulatory collapse, shock, vasodilation Treatments: cortisone, EpiPen</p> Signup and view all the answers

What is a type II hypersensitivity reaction?

<p>Cytotoxic that causes tissue damage and rheumatic fever (strep pyogenes) Complement: destroy red blood cell or enhance phagocytosis Antigen on plasma membrane on foreign cell, B cell get ready to make antibody, antibody bind to antigen, complement activated. IgM and IgG activate it and bind to antigen . The antibodies work against antigen on the surface of cell &amp; other tissue components and are destroyed by cytotoxic cells. deals with ABO blood group.</p> Signup and view all the answers

what is rheumatic fever?

<p>Caused by streptococcus pyogenes. Make antibodies against patient that go to the heart and causes cell destruction.</p> Signup and view all the answers

drug induced thrombocytopenia purpura steps

<p>Drug was binding with the platelet of pt, forming hapten platelet complex. Hapten can serve as an antigen hernia bound to a high molecular weight protein. This causes pt to make antibodies against their own platelets. The action of antibodies and complement causes destruction of platelets. These antibodies are autoantibodies since they are destroying the body’s own cells.</p> Signup and view all the answers

Type III hypersensitivity reaction?

<p>Diseases: serum sickness &amp; Arthus reaction. Antibody-antigen complexes deposit in blood vessels and basement membrane. The complexes is continuously formed. It leads to complement activation and acute inflammmation. Complex get engulfed by phagocytosis cells. Uses IgG and IgM.</p> Signup and view all the answers

What is a type IV hypersensitivity reaction?

<p>Cell mediated (T) response . Delayed hypersensitive that takes many hours to form it occurs when lymphokine released from cell. MIF prevent migration of macrophages so the remain localized at site of hypersensitivity reaction; Delayed: CD4 lymphocyte, class MHC2 extrinsic antigen, activate macrophage, PPD test Cell mediated: CD8 lymphocyte, intrinsic class 1 MHC complex antigen, viral infection, transplant rejection, autoimmune diseases.</p> Signup and view all the answers

What is immunosupression?

<p>Minimize immune reaction by impairing T cell responses. There are 3 main types Transplants: pt given immunosuppressive drug Radiation: destroy lymphoid function Cytotoxic: damage cell and interfere with DNa synthesis</p> Signup and view all the answers

Types of transplants?

<p>Autograft: tissue from 1 site of body to another (no response) Isograft: tissue form identical twin Allograft; diff individuals of same species (will be on antirejection meds) Xenograft; tissue exchange between individuals of different species.</p> Signup and view all the answers

Host rejection disease?

<p>Cytotoxic T recognize foriegn MHC on grafted cells and release IL2. Can be from humoral immune response and means of chronic rejection</p> Signup and view all the answers

3 types of host rejection?

<p>Hyperacute rejection which is mediated by performed antibodies specific for antigens on graft endothelial cells. acute rejection: mediated by T cells &amp; macrophages and activated by allioantigens in graft. Chronic rejection: indolent form of graft damage and causes progressive loss of graft function</p> Signup and view all the answers

Graft vs host disease (GVHD)?

<p>Occurs when the immunologically competent cells of thur recursions are trapped in immunologic depled recipients and the transferred cells find alloantigens. Seen in pt with transplants. Caused when the T cell in donor graft sees the host tissue as foreign and leads to CD4 and CD8 being activated. This leads to a depletion of T cells.</p> Signup and view all the answers

Different types of GVGD?

<p>Active: days-weeks after transplant and has epithelial cell necrosis in liver, skin, GI tract Chronic: follows acute or occurs instantly</p> Signup and view all the answers

What is autoimmune?

<p>Pt is hypersensitive to antigen or cels or tissues of heuristic own bodies despite mechanism they create to tolerate antigen. Cause an immune response where antibodies are produced. The adaptive immune response occurs against self-antigens and self tolerance causes harmful reactions to the host.</p> Signup and view all the answers

What is Systemic Lupus Erythematous (SLE)?

<p>Antibodies are made against nuclear antigens (against dsDNA). It is deposited in the basement of membranes and immune complex of binding antibodies to cells and tissues. It is a B cell mediated reaction where the body can’t recognize its own antibodies. It causes inflammation in skin, joints, kidneys. It also has the butterfly rash on the face. Occurs mostly in wome</p> Signup and view all the answers

What is rheumatoid arthritis?

<p>rheumatoid factor antibodies are deposited in the joints. This leads to connective tissue and synobial joint issues, like inflammation of the joints (syanitis). It very rare but seen in women. Rheumatoid factor (RF) Os an antibody that was first found, it directs IgM against the Fc region of IgG, causing RF and IgG to form immune complexes</p> Signup and view all the answers

what is Systemic Sclerosis/Scleroderma?

<p>It is a fibrosis of the skin &amp; walls of GI tract and vascular abnormalities. It causes excessive fibrosis and obliterate vascular disease and autoantibody. It’s hallmark is the deposition of collagen in organs and extracellular matrix. Autoantibodies cause platelet derived growth factors which causes development of fibrosis. It’s mostly seen in the skin. An ANA test can be used to reveal it which has a speckled pattern.</p> Signup and view all the answers

What is amyloidosis?

<p>A systemic disorder that is caused by deposition in tissues of insoluble proteins from a circulating precursor. It causes low molecular weight fibrils giving a “waxy” appearance to infiltrated organs, It has a primary amyloid light chain from light chains of plasma cells. It has a secondary amyloid-associated protein that is non immunoglobulin protein. It’s hallmark is showing an apple-green color in a Congo red stain</p> Signup and view all the answers

What is Cryoglobulinemia?

<p>Presence of serum of 1 oor more Ig below 37C. I has 3 types Type 1: single monoclonal Ig with no RFanf causes hypo proliferation malignancies of B cells. Type II: monoclonal IgM with polyclonal IgG and IgA in hepatic C pt Type III: mixed cwith polyclonal IgM RF with connective tissue damage and chromic illness Types 2 and 3 cause: hypoplementemia, complex vasculitis, arthritis, neuropathy, renal involvement</p> Signup and view all the answers

What is Sjogren Syndrome?

<p>Destruction of lacrimal and salivary glands leading to reduced tears and saliva. Causes symptoms such as dry eyes, dry mouth, and systemic connective tissue disease. Autoantibodies in connective tissue and inflammation of glands. Schirmer test detects less tears and lip biopsy diagnose t.</p> Signup and view all the answers

What is Myasthenia gravis?

<p>A neurodisorder of the muscles where autoantibodies are the acetylcholine receptor lines. Autoantibodies block the post synaptic membrane so acetylcholine can’t bind there, leading to paralysis. Acetylcholine as to get to the post synaptic membrane so it causes an electrical signal, leading to the muscles to contr</p> Signup and view all the answers

what are the primary immunodeficiency disorders?

<p>They are caused buy genetic defects that impact innate immunity. B cell defect: agammglobinemia, hypogammaglobinemia, selective immunological defiencies T cell defect: thymic aphasia, chemo mucutaneous candidiasis Both T and B: SCID, ADH Other: phagocyte and complement defects</p> Signup and view all the answers

What are the secondary immunodeficiency diseases?

<p>Caused by an infectious agent, HIV, malignancies, immunisupressants Natural sources: AIDS, nutrition deficiencies, stress, pregnancy, agin Immunosuppressive agents: irradiation, severe burns, steroids, chemotherapy, anti-rejection, removal of spleen</p> Signup and view all the answers

What is X-linked Agammaglobulinemia (Burton agammaglobulinemia)?

<p>It is a humoral deficiency with low antibodies and small tonsils. The B cells dont mature. It can be detected using CD19 and CD20. It occurs typicall in newborn baby males. They are asymptomatic for the first few month and then develop infections. It is caused by the loss of function of tyrosine kinase protein and protein BTK, which causes a defective maturation of B lymphocyte</p> Signup and view all the answers

what is common variable immunodeficiency?

<p>Its caused when the B cells are dysfunctional and they can’t secrete antibodies. It causes hypoglobinemia and malfunctions with IgG. It’s diagnosed using serum electrophoresis, serum IgG, IgM, cd19 and cd20. It has a normal B cell count in blood and lymph nodes but it is abnormal.</p> Signup and view all the answers

What is Hyper IgM syndrome?

<p>It is caused by reduced IgG and IgA with normal IgM and circulating B cells. it is caused by X-linked autosomal recessive mutation which causes to T cells to not activate B cells and macrophages. It can also be caused by cellular causes such as S-linked loss of function. Increased IgM causes polyclonal expansion of IgM synthesis in response to infection from encapsulated becateria.</p> Signup and view all the answers

What is selective IgA deficiency?

<p>It is caused by B-cell differentiation that arrest in IgG to IgA isotope switch. It has low IgA bearing B cells. It cauases anaphylaxis from products with IgA. It causes allergies, autoimmune disorders. It is diagnosed when IgA level is less than 5 mg/dL.</p> Signup and view all the answers

What is severe combine immunodeficiency Syndrome (SCID)?

<p>It is caused by T and B cells from defective IL-2 receptors. The x linked form is Connor in the gamma chain and the autosomal recessive form is the Swiss type that lacks adenosine delaminate. It manifests as widespread infection after birth and respiratory failure.</p> Signup and view all the answers

Deficiency of complement proteins?

<p>C3 deficiency: inc ability to get infections of encapsulated pyogenes bacteria C5,C6,C7C9: neisseira infections C1q: scavenges DNA from apoptosis C2 and C4: autoimmune diseases C1: defect in SERPINGI and hereditary audio edema</p> Signup and view all the answers

What is DiGeorge Syndrome?

<p>Defective development of pharyngeal pouches 3 and 4 from deletion of chromosome 22. Lack of thymus leads to no T cells causing infections. Lack of parathyroid gland leads to hypercelcemia ad tetary. It causes CATCH22 (cadairc abnormality, abnormal fancies, hypertelorism, low set ears). Cleft palate it infects</p> Signup and view all the answers

What is wisdom Aldrich syndrome (WAS)?

<p>It is caused by a mutation in a gene that codes for wisdom-Aldrich Syndrome protein (WASP). It causes B &amp; T cell deficiency, low IgM, high IgA, normal IgE. It causes pyogenes infections, eczema, thrombocytopenia.</p> Signup and view all the answers

What is Acquired Immunodeficiency Syndrome (AIDS)?

<p>Caused from HIV, an infection of CD$ lymphocytes. It is an inversion of CD4 and CD8 ration which is normall 2:1. It causes decreased humoral and cell mediated immunity. It causes increased chance of malignancy and recurrent infection</p> Signup and view all the answers

What is Hashimoto Thyroiditis?

<p>autoimmune disorder where the immune system attacks the thyroid gland. It is often genetically predisposed and can be triggered by environmental factors like infections or stress. The thyroid is gradually damaged by immune cells and antibodies.</p> <p>Main Symptoms:</p> <p>Fatigue Weight gain Cold intolerance Constipation Dry skin and hair Depression Enlarged thyroid (goiter) Slow heart rate (bradycardia) Pathogenesis: The immune system produces antibodies that target the thyroid gland, particularly the thyroid peroxidase (TPO) enzyme. This leads to inflammation, glandular damage, and reduced thyroid hormone production (hypothyroidism). As the thyroid becomes less functional, it can no longer regulate metabolism effectively, leading to the symptoms of hypothyroidism.</p> Signup and view all the answers

What is type 1 diabetes?

<p>ause: Type 1 diabetes is an autoimmune disorder where the body's immune system attacks and destroys the insulin-producing cells in the pancreas, known as beta cells. The exact cause is unknown, but genetic and environmental factors, such as viral infections, are thought to play a role in triggering the immune response.</p> <p>Main Symptoms:</p> <p>Increased thirst (polydipsia) Frequent urination (polyuria) Extreme hunger (polyphagia) Unexplained weight loss Fatigue Blurred vision Slow healing of cuts or infections Pathogenesis: In Type 1 diabetes, autoimmune destruction of the beta cells in the pancreas leads to insulin deficiency. Insulin is a hormone that helps the body use sugar (glucose) for energy. Without insulin, glucose builds up in the blood (hyperglycemia) and cannot be effectively used by cells for energy. This results in the hallmark symptoms and complications of diabetes, such as high blood sugar, energy loss, and potential long-term damage to organs.</p> Signup and view all the answers

Flashcards

Hypersensitivity

An antigenic response that is beyond what is considered normal.

Allergy

A condition of altered reactivity or exaggerated immune response, often manifested by inflammation.

Type 1 Hypersensitivity

IgE antibodies bind to mast cells and basophils, causing degranulation and release of chemical mediators upon subsequent antigen exposure, leading to anaphylaxis.

Type 1 Hypersensitivity Sequence

B cell recognizes allergen, activates, and produces IgE. IgE binds to mast cells/basophils. Subsequent exposure causes degranulation and release of chemical mediators, leading to anaphylaxis.

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RAST Test

A blood test for allergy testing; tests the blood, not the patient. Safer but less sensitive than skin tests.

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Conditions of Hypersensitivity 1

Chronic local allergy (Atopy) and systemic explosive reaction with airway obstruction, circulatory collapse, shock, and vasodilation (Anaphylaxis).

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