Patho Ch. 12

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1
humoral immunodeficiencies
What are the b-cell function and immunoglobulin or antibody productionar
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immunodeficiency
an abnormality in one or more parts of the immune system that results in an increased susceptibility to disease states normally eradicated by the properly functioning immune response
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primary immune deficiencies
Deficiency of the immune system that results from improper development of immune cells or tissues; usually congenital or inherited
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secondary immune deficiencies
Immunodeficiency in which b-cell function and immunglobulin or antibody production are involved
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recurrent bacterial infections and pyrogenic infections
What risks are increased with humoral immunodeficiencies?
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Transient hypogammaglobulinemia of infancy
humoral immunodeficiency that is usually normal by 11 months. Low IgG, normal IgA, variable IgM:
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70%
Approximately what percent of primary immunodeficiencies are humoral?
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one or all of them at the same time
Which immunoglobulins are affected with primary humoral immunodeficiency disorders?
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DiGeorge syndrome
a primary immunodeficiency, often but not always, characterized by cellular (T-cell) deficiency, characteristic looks, congenital heart disease, and hypocalcemia; caused by abnormal formation of certain tissues during fetal development; PARTIAL OR COMPLETE FAILURE OF THYMUS AND PARATHYROID DEVELOPMENT
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acute viral syndromes
What are secondary cell mediated immunodefiencies often associated with as a cause?
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ataxia-telangiectasia
a combined SCID (severe combined immunodeficiency) that is an autosomal recessive multisystem disorder with cerebellar neurodegeneration and ataxia: deficiency in t-cells, IgA, IgE, and IgG
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wiskott-aldrich syndrome
a combined SCID (severe combined immunodeficiency) that is an x-linked disorder characterized by thrombocytopenia, recurrent infections, and eczema; life expectancy 15 years; low levels of IgM and increased levels of IgA and IgE
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Leukemia, Hodgkin lymphoma
Risk for which cancers increase with diagnosis of wiskott-aldrich syndrome?
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Stem cell or bone marrow transplant
What is the treatment for t-cell and b-cell immunodeficiencies?
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hereditary angioneurotic edema
primary disorder of the complement system that causes a loss of regulation in release of vasoactive substances that promote vascular permeability causing deep localized tissue swelling
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phagocytic system
immune system that is composed primarily of polymorphonuclear leukocytes and mononuclear phagocytes
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mycobacteria (intracellelular bacteria); yeast
What are 2 examples of types of pathogens that are increased risk for with phagocytic immunodeficiency?
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the phagocytic system also acts as a bridge between the innate and adaptive system
Why could the adaptive and innate immune systems be compromised if there is phagocytic immunodeficiencies?
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hypersensitivity disorder
excessive or inappropriate activation of the immune system
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type I
IgE mediated hypersensitivity disorder
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type II
antibody mediated hypersensitivity disorder
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type III
complement mediated hypersensitivity disorder
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type IV
T-cell mediated hypersensitivity disorder
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atopic disorders
heredity predisposition and production of a local reaction to IgE antibodies produced in response to common environmental agents
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urticaria (hives), allergic rhinitis, atopic dermatitis, food allergies, some forms of asthma
What are the symptomatic presentations of Type 1 (IgE mediated) allergic reactions?
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allergy shots (desensitization), cromolyn sodium, nasal steroids, avoidance
What are the ways to manage Type I (IgE mediated) allergic reactions?
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peanuts, tree nuts, shellfish
What are the most common food allergies?
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vasodilation, vascular leakage, smooth muscle contraction
What occurs in the primary or initial phase response of Type 1 hypersensitivity reactions (IgE mediated)?
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degree of sensitization
What does the severity of a Type I reaction depend on?
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4
How many grades on the continuum of severity for Type I reactions are there?
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grade I
grade of type 1 hypersensitivity reaction in which there is cutaneous and mucosal erythema and urticaria
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grade II
grade of type 1 hypersensitivity reaction in which there is multisystem signs; hypotension, tachycardia, dyspnea, GI disturbance (n/v/d)
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grade III
grade of type 1 hypersensitivity reaction in which there is bronchospasms and cardiac dysrhythmias
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grade IV
grade of type 1 hypersensitivity reaction in which there is cardiac arrest
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anaphylaxis
the most severe for of type I hypersensitivity reaction in which there is widespread release of histamines
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maintain airway, volume expansion (fluids), epinephrine
What are the treatments for anaphylaxis?
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IgG or IgM
Which antibodies mediate type II (cytotoxic) hypersensitivity reactions?
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mismatched blood tranfusion reactions, hemolytic disease of the newborn
What are some examples of type II (cytotoxic) hypersensitivity?
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insoluble antigen-antibody complex that activate the complement pathway
what mediates the type III immune complex disorders?
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alterations in blood flow, increased vascular permeability, destructive action of inflammatory cells
How do the results of the complement system in type III hypersensitivity reactions cause tissue damage?
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kidney, skin, lung, joints, vasculitis
What parts of the body do type III immune complex allergic disorders affect?
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serum sickness; 1-2 weeks
What is an example of systemic type III immune complex allergic disorders and how long does it take to manifest?
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arthus reaction (discrete tissue necrosis, red, raised, inflamed); within 1 hour
What is an example of localized type III immune complex allergic disorders and how long does it take to manifest?
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direct and delayed-type
What are the 2 basic types of type IV hypersensitivity reactions?
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hepatitis
What is an example of direct cell-mediated cytotoxicity?
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allergic contact dermatitis, hypersensitivity pneumonitis
What are 2 examples of delayed-type hypersensitivity?
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CD8+ T-cells
Which cell line is responsible for direct cell mediated Type IV hypersensitivity reaction?
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CD4+ T-cells
Which cell line is responsible for delayed-type Type IV hypersensitivity reaction?
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type I and type IV
What types of hypersensivity reactions are involved in latex sensitivity?
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major histocompatibility complex
What does MHC stand for?
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MHC
set of molecules displayed on all cell surfaces that control the immune response through recognition of "self" and "non-self" by recognizing lymphocytes and antigens
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allogeneic, syngeneic, autologous
What are the 3 categories of transplanted tissues?
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allogeneic
type of tissue transplant in which the donor and recipient are related or unrelated but share similar HLA types
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syngeneic
type of transplanted tissue in which the donor and recipient are identical twins
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autologous
type of transplanted tissue in which the donor and recipient are the same person
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bone marrow, peripheral blood, umbilical cord blood
Where can stem cells be pulled from for stem cell transplants?
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self-tolerance
the ability to differentiate between foreign antigens and self-antigens
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central tolerance
The elimination of self-reactive T cells and B cells in the central lymphoid organs
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peripheral tolerance
Derives from the deletion or inactivation of autoreactive T cells or B cells that escaped elimination in the central lymphoid organs
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hyperacute rejection
transplant rejection that occurs almost immediately after transplantation; a type III arthus type reaction
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acute rejection
transplant rejection that occurs within the first few months after transplantation with signs of organ failure; T-lymphocytes respond to antiges in the graft tissue
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chronic host-versus-graft rejection
transplant rejection that occurs over a prolonged period and manifests with dense intimal fibrosis of blood vessels of the transplanted organ
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graft versus host reaction
An attack against a patient's body cells by the transplanted tissue or organ
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the transplanted tissue must have a functional cellular immune component; the recipient immunity must be compromised
What are the basic requirements necessary for graft versus host disease?
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autoimmune diseases
A heterogeneous group of disorders that occur when the body's immune system fails to differentiate "self" from "non-self" and mounts an immunologic response against host tissues
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SLE (systemic lupus erythematosus)
an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.
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AIHA (autoimmune hemolytic anemia)
occurs when antibodies directed against the person's own red blood cells cause them to burst (lyze) leading to insufficient plasma concentration
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pemphigus vulgaris
a rare and serious condition that causes painful blisters to develop on the skin and lining of the mouth, nose, throat, and genitals. The blisters are fragile and can easily burst open leaving areas of raw unhealed skin that are very painful and can put you at risk for infections
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hashimoto thyroiditis
an autoimmune disease in which the thyroid gland is attacked by a variety of cell and antibody mediated immune processes, causing primary hypothyroidism. It was the first disease to be recognized as an autoimmune disease.
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viral infections, chemical substances (smoking), previously hidden self-antigen
What are the trigger events for autoimmune disease?
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ELISA, IFA, particle agglutination assay
What are the lab tests used to demonstrate antibodies against tissue antigens or cellular components?
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enzyme-linked immunosorbent assay
What does ELISA stand for?
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indirect fluorescent antibody assay
What does IFA stand for?
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reverse transcriptase
An enzyme encoded by some certain viruses (retroviruses) that uses RNA as a template for DNA synthesis.
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category 3 or category c
What category must a HIV+ patient be classified in to have an AIDS diagnosis?
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> 500
What is the t-cell count in a HIV+ patient classified as category 1?
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200-499
What is the t-cell count in a HIV+ patient classified as category 2?
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< 200
What is the t-cell count in a HIV+ patient classified as category 3?
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category a
The clinical category for a HIV+ patient that is asymptomatic or has persistent generalized lymphadenopathy
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category b
The clinical category for a HIV+ patient that has symtpoms of immune deficiency but not serious enough to be AIDS defining
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category c
The clinical category for a HIV+ patient that has AIDS defining illnesses
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mono like illness
What occurs during the primary infection phase of HIV?
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no symptoms or lymphadenopathy
What occurs during the asymptomatic or latency phse of HIV?
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immunosuppression, malignancies, wasting, opportunistic infections, CNS degeneration
What are the characteristics of AIDS?
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10-11 years after infection
How quickly will HIV become AIDS in untreated typical progressors?
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< 5 years
How quickly will HIV become AIDS in untreated rapid progressors?
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> 15 years
How quickly will HIV become AIDS in untreated slow progressors?
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infected for at least 8 years, have high CD4+ counts, have low viral loads, are antiretroviral naive
What are the qualifications for considering an untreated HIV patient a long-term nonprogressor?
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bacterial pneumonia, MAC, TB, salmonella bacteremia
What are some examples of bacterial opportunistic infections found in AIDS patients?
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candidiasis, coccidiomycosis, cryptococcosis, and histoplasmosis
What are some examples of fungal opportunistic infections found in AIDS patients?
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cryptosporidiosis, isosporiasis, pneumocystiasis, toxoplasmosis
What are some examples of protozoal opportunistic infections found in AIDS patients?
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cytomegalovirus (CMV), JC virus, herpes, progressive multifocal leukoencephalopathy (PML)
What are some examples of viral opportunistic infections found in AIDS patients?
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AIDS dementia complex (ADC), toxoplasmosis, PML (progressive multifocal leukoencephalopathy)
What are some nervous system manifestations associated with AIDS?
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kaposi sarcoma, non-hodgkin lymphoma, noninvasive cervical carcinoma
What are some cancers and malignancies associated with AIDS?
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wasting syndrome
Involuntary weight loss of at least 10% of baseline body weight, presence of diarrhea, more than two stools per day, chronic weakness, and fever
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ELISA, western blot, PCR (polymerase chain reaction)
What are the lab testing methods for HIV infection?
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ELISA
screening test to detect anti-HIV antibodies in the bloodstream
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high false positive rates
What is the main problem with the ELISA test for HIV?
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Western blot test
a blood test that produces more accurate results than the ELISA test that looks for antibodies to SPECIFIC viral antigens
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Orasure test
uses a swab of the cheek to perform an EIA antibody test
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PCR (polymerase chain reaction)
A laboratory technique for amplifying DNA in vitro by incubating with special primers, DNA polymerase molecules, and nucleotides; detects HIV DNA - not antibodies to the virus; useful in testing infants born to infected mothers
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viral load, CD4+ counts, opportunistic infections
Therapeutic interventions and frequency of follow-up for HIV are determined by the level of disease activity based on:_________________
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HAART (highly active antiretroviral therapy)
Combination of drugs effective against AIDS; used in the treatment of HIV
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reverse transcriptase inhibitors
HIV medication that acts by blocking the elongation of the DNA chain by stopping the addition of nucleosides by blocking REVERSE TRANSCRIPTASE
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Protease inhibitors
HIV medication that binds to proteases and inhibit their function
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fusion inhibitors
HIV medication that prevents viruses from successfully fusing with the host cell
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test when they become pregnant AND prior to labor
What are the recommendations for testing pregnant women for HIV?
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zidovudine/lamivudine
What is the recommended antiretroviral therapy for women who are pregnant?
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efavirenz
What HIV antiretroviral should *not* be given to pregnant patients in the *first trimester* due to neural tube defects?
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in utero, during labor and delivery, through breast feeding
When can HIV be passed from mother to infant?
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6 months
How long does the maternal IgG antibody for HIV remain in the infant that prevents accurate testing of the infant?
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18 months
How long may an infant be HIV+ by ELISA for due to maternal antibodies?
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failure to thrive, CNS abnormalities, develpment delays
How does HIV present in children?
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pneumocystis jiroveci pneumonia (PCP)
What disease is a major cause of mortality in HIV+ children that presents early in infants but late in adult infection?
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immaturity of the liver
Why do we wait until 4-6 weeks of age to treat infants prophylactically with Bactrim?
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