I2: Clinical Correlates

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xenotransplantation from pigs is particularly susceptible to membrane attack because

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pic complement regulatory proteins do not regulate human complement components

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Factor I deficiency affects

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the positive feedback amplification loop of the alternate pathwaya

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antibody -mediated therapy for cytokine mediated inflammatory diseases does what

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Ig molceules bind to cytokine receptors

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Ig locus deletions leads to

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normal B cell number but no IgG1, IgG2, IgG4 or IgA1

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Do Ig deletions cause increased susceptibility to infection

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no

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SCID is a pediatric______

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emergency

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chromosomal translocations to Ig loci could cause

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malignant lmphoid cells

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Biclonal myeloma occurs because

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commitment to a particular H chain class occurs later, providing an opportunity for those rare multiple isotypic neoplasias to arise

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Biclonal myeloma characteristics of Ig

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different class
but identical V region sequences

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1st pregnancies are not effected by Rh disease becauuse

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IgM Ab are present and these do not cross the placenta

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Rh disease occurs in the second pregnancy because

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IgM switches to IgG

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Coombs test detects

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IgGs on surface of neotnates RBCs

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hypergammaglobulinemia can be caused by

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repeated immunization, infection, abnormal B cell differentiation

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benign monoclonal gammopathy of undetermined significance usually occurs in

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elderly

Front 15

what determines if gammopathies are monoclonal/polyclonal

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immunofixation

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CD3 deficiency leads to the inability to

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signal through pre-TCR

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positive selection needs

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pre-TCR

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abnormal BCR expression treatment

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antibiotics for infections

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few peripheral T cells could indicate ____or_____

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viral/fungal infections
autoimmunity

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penicillin-specific Ab can bind___ and induce ____

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RBC and hapten and induce autoimmune hemolytic anemia

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if there is a coombs positive test-

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discontinue drug and transfuse normal ABO-matched RBC

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antibodies to polysaccharide capsules =

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opsonins

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multiple myeloma can lead to

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pancytopenia

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why wouldn't you give a multiple myeloma patient IVIG

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the circulating levels of IgM are high and therefore there would be an elevated rate of clearance for antibodies in IVIG

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rhogam delivers an inhibitory signal for B cell activation through

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intracellular SHIP molecule

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trophoblastic cells that form the fetal interface with maternal circulation are

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devoid of most HLA antigens

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trophoblast cells also do not produce

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B7

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____ are expressed at high levels at sites of implantation

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Inhibitory factors, IL-10, Inhibitory B7 isoforms, death receptor ligands and PGE2

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Rheumatic fever affects ___aged children and is caused by

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school age caused by streptococcus pyogenes

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rheumatic fever affects the

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heart, joints, and CNS

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oral thrush is umcommon in

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immunocompetent individuals

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oral thrush is caused by

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candida albicans- yeast

Front 33

A similar phenotype of X-linked agammaglobulinemia exists in patients with

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H locus deletions that prevent functional m chain synthesis and pre-BCR expression

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AID

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recombinase called activation-induced cytidine deaminase

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mutations in AID

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imparied antigen-induced isotype switching, somatic hpyermutation in B cells and affinity maturation of ab response

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allergic asthma could theoretically be treated by

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blocking B cell switching to IgE synthesis within anti-IL-4 or anti-IL-13

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IgG2 is commonly for

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T-independent antigens

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Many CD patients have mutations in the

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NOD2 antimicrobial protein which diminishes their ability to clear translocated bacteria

Front 39

all of these can activate mast cells

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drugs
anaphylatoxins
neuropeptides substance P and somatostatin
Lectins

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key target organs of systemic allergic anaphylaxis

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cardiovascular, pulmonary, gastrointestinal and cutaneous

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eosinophils

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damage the mucosal epithelium, cause perivascular edema and initiate lung tissue remodeling

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mucus plugging of the bronchi results in

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hyperinflation of the lungs, an inability to expire air and expiratory wheezing

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what increases the risk in children of developing IgE-mediated food allergies

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inability to secrete mucosal IgA

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serum sickness in immune individuals typically becomes evident

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1-3 days after exposure to the antigen

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signs and symptoms of serum sickness

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fever, rash, hypotension, anaphylactoid purpura, lymphagenopathy, myalgia, arthralgia and nephritis

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when the fetus has____ GVGD can occur

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congenital T cell deficiencies

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is increased incidence of infection a consistent feature of autoimmune diseases?

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no

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what can impair host responses to infectious agents in autoimmune diseases

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complement consumption