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84 Cards in this Set
- Front
- Back
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Bruton's agammaglobulinemia defect
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Bruton's agammaglobulinemia defect:
X-linked -defect in BTK, a tyr kinase gene, blocks B-cell differentiation/maturation |
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Bruton's agammaglobulinemia presentation & labs
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Bruton's agammaglobulinemia presentation & labs:
Presentation -recurrent bacterial infections after 6mo old b/c bad opsonization Labs: -nrl pro-B, but low maturation, number of B cells, low Ig (all Ig) -defect in BTK (try kinase) blocks B cell diff & maturation |
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Hyper-IgM syndrome defect
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Hyper-IgM syndrome defect:
-defective CD40L on Th; results in inability to class switch |
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HyperIgM syndrome presentation & labs
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HyperIgM syndrome presentation & labs
Presentation -severe pyogenic infections early in life Labs -high IgM, Low IgG, A, & E -defective CD40L doesn't allow class switching |
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Selective Ig deficiency defect
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Selective Ig deficiency defect
-defect in isotype switching leads to deficiency in specific class of Ig |
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Selective Ig deficiency presentation & labs
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Selective Ig deficiency presentation & labs
Presentation: -sinus & lung infections, milk allergies, diarrhea -anaphylaxis on exposure to blood products w/ IgA Labs: -deficient IgA is most common defect in isotype switching |
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Common Variable Immunodeficiency (CVID) defect
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Common Variable Immunodeficiency (CVID) defect
-defect in B cell maturation (many causes) |
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Common Variable Immunodeficiency (CVID) presentation & labs
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Common Variable Immunodeficiency (CVID) presentation & labs
Presentation: -can be acq'd in 20's-30's -incr risk of autoimmune disease -lymphoma, sinopulmonary infections Labs: -nrl [B cells], low plasma cells, low Ig -defect in B cell maturation |
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Thymic Aplasia (DiGeorge Syndrome) defect
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Thymic Aplasia (DiGeorge Syndrome) defect
-22q11 deletion -failure to develop 3rd & 4th Pharyngeal Pouches |
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Thymic Aplasia (DiGeorge Syndrome) presentation & labs
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Thymic Aplasia (DiGeorge Syndrome) presentation & labs
Presentation: -tetany (hypocalcemia) -recurrent viral/fungal infections (T-cell deficiency) -congenital heart & great vessel defects Labs: -Thymus & Parathyroids fail to develop -leads to decreased: T cells, PTH, Calcium -absent thymic shadow on CXR 22q11 deletion, 3rd & 4th pharyngeal pouches don't develop |
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IL-12-Receptor deficiency defect
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IL-12-Receptor deficiency defect
-defect in Th1 response |
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IL-12-R deficiency presentation & labs
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IL-12-R deficiency presentation & labs
Presentation: -disseminated mycobacterial infections -low IFN-gamma -low Th1 response |
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Hyper-IgE syndrome (Job's syndrome) defect
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Hyper-IgE syndrome (Job's syndrome) defect
-Th cells fail to make IFN-gamma, results in inability of neutrophils to respond to chemotactic stimuli |
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Hyper-IgE syndrome (Job's Syndrome) presentation & labs
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Hyper-IgE syndrome (Job's Syndrome) presentation & labs
Presentation: -FATED: coarse Facies, cold (noninflamed staph Abscesses, retained primary Teeth, high IgE, Dermatologic problems (ie eczema) Labs: -High IgE -Th don't make IFN-gamma, neutrophils can't respond |
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Chronic Mucocutaneous Candidiasis defect & presentation
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Chronic Mucocutaneous Candidiasis defect & presentation
Defect: -T-cell dysfunction Presentation: -Candida infections of skin & mucous membranes |
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Severe Combined Immunodeficiency (SCID) defect
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Severe Combined Immunodeficiency (SCID) defect
-many; m/c is X-linked defective IL-2-R -adenosine deaminase defic -failure to make MHC-II Ag |
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Severe Combined Immunodeficiency (SCID) presentation & labs
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Severe Combined Immunodeficiency (SCID)
Presentation: -recurrent viral, bacterial, fungal, & protozoal infections due to BOTH B & T cell defic -Tx = Bone Marrow Transplant Labs: -low IL-2-R = low T-cell activation -high adenine = Toxic to B & T cells -low dNTPs, low DNA synthesis |
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Ataxia-Telangiectasia defect
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Ataxia-Telangiectasia defect
-defect in DNA repair enzymes |
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Ataxia-Telangiectasia presentation & labs
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Ataxia-Telangiectasia
Presentation: Triad: cerebella defects (ataxia), spider angiomas (telangiectasia), IgA deficiency Labs: -IgA deficiency |
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Wiskott-Aldrich Syndrome defect
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Wiskott-Aldrich Syndrome defect
-X-linked recessive -progressive deletion of B & T cells |
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Wiskott-Aldrich Syndrome Presentation & Labs
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Wiskott-Aldrich Syndrome
Presentation: -Triad (TIE): Thrombocytopenic purpura, Infections, Eczema Labs: -High IgE, High IgA -Low IgM |
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Leukocyte Adhesion deficiency (type 1) defect
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Leukocyte Adhesion deficiency (type 1) defect
-defect in LFA-1 Integrin (CD18) on phagocytes |
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Leukocyte Adhesion deficiency (type 1) presentation & labs
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Leukocyte Adhesion deficiency (type 1)
Presentation: -recurrent bacterial infections, absent pus formation, delayed separation of umbilicus Labs: -Neutrophilia |
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Chediak-Higashi Syndrome defect
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Chediak-Higashi Syndrome defect
-autosomal recessive -defect in microtubular function w/ deficient phagocytosis |
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Chediak-Higashi Syndrome presentation
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Chediak-Higashi Syndrome
Presentation: recurrent infections by staphylococci & streptococci -partial albinism -peripheral neuropathy *defect in microtubular dysfunction w/ decr phagocytosis |
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Chronic Granulomatous Disease defect
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Chronic Granulomatous Disease defect
-lack of NADPH Oxidase -leads to decr Reactive O2 Species & absent Respiratory Burst in Neutrophils |
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Chronic Granulomastous Disease presentation & labs
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Chronic Granulomastous Disease
Presentation -incr susceptibility to catalase-positive organsims (like S aureus, E coli, Aspergillus) Labs: -Negative Nitroblue Tetrazolium dye reduction test |
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How does Cyclosporine work?
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How does Cyclosporine work?
-by binding Cyclophilins, the complex inhibits Calcineurin -Calcineurin is req'd for activated T cell to produce IL-2 & IL-2R -thus, T cells can't activate completely -for organ rejection suppression |
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Cyclosporin toxicity?
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-predisposes to viral infections & lymphoma
-nephrotoxic, but prevent w/ Mannitol diuresis -remember, blocks Calcineurin from allowing IL-2 & IL-2R production |
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how does Tacrolimus (FK506) work?
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how does Tacrolimus (FK506) work?
-like Cyclosporin, but instead of binding Calcineurin (cyclosporine), this binds FK-binding protein -this inhibits IL-2 production -for immunosuppresion in organ transplant |
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SE of Tacrolimus
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SE of Tacrolimus
signifiant: Nephrotoxicity, Peripheral Neuropathy, HTN, Pleural Effusion, Hyperglycemia -remember, it binds FK-binding protein to block IL-2 production |
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Azathioprine, how does it work?
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Azathioprine, how does it work?
-anti-metabilite, precursor to 6-Mercaptopurine -thus, interferes w/ metabolism & synthesis of nucleic acids (esp in proliferating lymphocytes) -for kidney transplant, autoimmune d/o |
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SE of Azathioprine
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SE of Azathioprine
-Marrow Suppression -active metabolite (Mercaptopurine) is metabolized by Xanthine Oxidase, thus toxic effects may be incr by Allopurinol -remember, its a precursor to 6-Mercaptopurine that interferes w/ nucleic acid synthesis |
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how does Muromonab-CD3 work?
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how does Muromonab-CD3 work?
-Monoclonal Ab that binds CD3 on T cell surface -blocks cell interaction w/ CD3 that's req'd for T-cell signal transduction -for immunosuppresion after kidney transplant |
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SE of Muromonab-CD3?
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SE of Muromonab-CD3?
-Cytokine Release Syndrome, Hypersensitivity rxn |
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how does Sirolimus (rapamycin) work?
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how does Sirolimus (rapamycin) work?
-binds mTOR, inhibits T-cell proliferation in response to IL-2 -immunosuppresion for kidney transplant (w/ Cyclosporine & Corticosteroids) |
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how does Mycophenolate Mofetil work?
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how does Mycophenolate Mofetil work?
-inhibits de novo Guanine synthesis, blocks lymphocyt production |
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how does Daclizumab work?
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how does Daclizumab work?
-Monoclonal Ab w/ high affinity for the IL-2R on activated T cells |
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what can Splenic dysfunction lead to (re type of infection)?
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what can Splenic dysfunction lead to (re type of infection)?
-decr IgM, leads to decr complement activation, leads to decr C3b opsonization, leads to incr susceptibility to: Encapsulated organisms (S pneumoniae, H influenzae, Salmonella, N meningitidis) |
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3 things a Peripheral Blood Smear will show post-splenectomy (or severe dysfunction)?
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3 things a Peripheral Blood Smear will show post-splenectomy (or severe dysfunction)?
-Howell-Jolly bodies on RBCs -Target Cell RBCs -Thrombocytosis |
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explain Positive vs Negative Selection re T-cells
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explain Positive vs Negative Selection re T-cells:
-(+) Selection = allowing T-cells that DO recognize self-MHC to survive -(-) Selection = destruction of T cells that DO recognize self-Ag -DO want T-cells to recognize self-MHC, do NOT want T-cells that recognize self-Ag |
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some differences btw Innate & Adaptive Immunity
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Innate Immunity:
-NO memory -rapid & nonspecific -Neutrophils, Macrophages, Dendritic Cells, NK cells, Compliment Adaptive Immunity: -V(D)J recomb -slow in 1st exposure, faster & more robust memory response -T-cells, B-cells, Ab |
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all T-cells express?
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all T-cells express?
CD3 |
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what stimulates a CD4+ cell to become Th1 vs Th2?
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what stimulates a CD4+ cell to become Th1 vs Th2?
-CD4+ exposed to IL-12 will become Th1 -CD4+ exposed to IL-4 will become Th2 |
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product of Th1 vs Th2 cells? & what inhibits them
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product of Th1 vs Th2 cells?
Th1: -make IL-2, IFN-gamma to activate macrophages & CD8+ Th2: -make IL-4, IL-5, IL-10 -IL-10 (from Th2) will inhibit Th1, IFN-gamma (from Th1) will inhibit Th2 |
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what causes Bear Lymphocyte Syndrome?
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no beta2-microglobulin (that nrl'ly complexes w/ MHC-I)
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-genes for MHC-I?
-expressed on |
genes for MHC-I?
-HLA-A, HLA-B, HLA-C expressed on? -all nucleated cells (so NOT on RBCs) |
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HLA-B27 a/w?
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HLA-B27 a/w?
-Psoriatic arthritis, Ankylosing Spondylitis, Inflamm Bowel Disease, Reiter's Syndrome (reactive arthritis) |
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HLA-A3 a/w?
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HLA-A3 a/w?
-Hemochromatosis |
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HLA-B8 a/w?
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HLA-B8 a/w?
-Graves disease |
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HLA-DR2 a/w?
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-MS, Hay Fever, SLE, Goodpasture's
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Type 1 DM is a/w which HLA-haplotypes?
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Type 1 DM is a/w which HLA-haplotypes?
-HLA-DR3 & -DR4 |
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HLA-DR4 a/w?
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HLA-DR4 a/w?
-RA & T1DM |
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HLA-DR5 a/w?
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HLA-DR5 a/w?
-Pernicious anemia, Hashimoto's Thyroiditis |
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HLA-DR7 a/w?
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HLA-DR7 a/w?
-Steroid-responsive Nephrotic syndrome |
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3 main APCs in immune system?
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3 main APCs in immune system?
-Macrophages -B cells -Dendritic cells |
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2 signals req'd for Th-cell activation?
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-foreign body phagocytosed by APC
-foreign Ag is presented on MHC II & recognized by TCR on Th cell (1ST signal) -"Co-stim Signal" given by B7/CD28 interaction (2ND signal) -Th cell is now activated to produce cytokines |
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2 signals req'd for Tc-cell activation?
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2 signals req'd for Tc-cell activation?
-endogenously synthesized (viral or self) proteins presented on MHC I & recognized by TCR on Tc cell (1ST signal) -IL-2 from Th cell activates Tc cell to kill virus-infected cell (2ND signal) |
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2 signals req'd for B cell class switching
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2 signals req'd for B cell class switching
-IL-4, IL-5, or IL-6 from Th2 cell is 1ST signal -CD40R activation by binding CD40L on Th cell is 2ND signal |
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how can Cimetidine increase cellular immunity?
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how can Cimetidine increase cellular immunity?
-blocks H2-R on T cells, leads to decr cAMP, leads to incr T cell activity |
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which is the Amino Terminal & which is the Carboxy Terminal in an antibody?
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which is the Amino Terminal & which is the Carboxy Terminal in an antibody?
-Amino Terminal = Fab portion (the Ag binding portion) -Carboxy Terminal = Fc portion (the Complement binding side) |
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the pyrogenic cytokines incl?
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the pyrogenic cytokines incl?
-IL-1, IL-6, TNF-alpha |
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cytokine that serves as major chemotactic factor for Neutrophils?
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cytokine that serves as major chemotactic factor for Neutrophils?
-IL-8 |
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differences btw IL-10 & IFN-gamma?
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differences btw IL-10 & IFN-gamma?
-IL-10 from Th2 cells -IL10 activates Th2, inhibits Th1 -IFN-gamma from Th1 cells -IFN-gamma inhibits Th2, activates Th1 |
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what do Decay-Accelerating Factor (DAF) and C1 esterase do?
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what do Decay-Accelerating Factor (DAF) and C1 esterase do?
-help prevent Complement activation on self-cells -defic of DAF leads to Paroxysmal Nocturnal Hemoglobinuria by Complement-med'd RBC lysis |
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4 instances when should give Passive immunity?
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4 instances when should give Passive immunity?
"To Be Healed Rapidly" -Tetanus, Botulinum, HBV, Rabies |
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anti-dsDNA, anti-Smith Abs
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anti-dsDNA, anti-Smith
-specific for SLE |
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anti-histone-Ab
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anti-histone-Ab:
Drug-induced lupus |
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anti-IgG (RF)-Ab
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anti-IgG (RF)-Ab:
Rheumatoid Arthritis |
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anti-centromere-Ab
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anti-centromere-Ab:
Scleroderma (CREST) |
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anti-Scl-70, anti-DNA Topoisomerase I Ab
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anti-Scl-70, anti-DNA Topoisomerase I Ab:
-Scleroderma (diffuse) |
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anti-mitochondrial-Ab
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anti-mitochondrial-Ab:
-Primary Biliary Cirrhosis |
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anti-gliadin, anti-endomysial Ab
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anti-gliadin, anti-endomysial Ab
Celiac disease |
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anti-basement membrane Ab
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anti-basement membrane Ab:
Goodpasture's syndrome |
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anti-desmoglein Ab
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anti-desmoglein Ab:
Pemphigus vulgaris |
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antimicrosomal Ab, antithyroglobulin Ab
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antimicrosomal Ab, antithyroglobulin Ab:
Hashimoto's thyroiditis |
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anti-Jo-1 Ab
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anti-Jo-1 Ab:
Polymyositis, Dermatomyositis |
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anti-SS-A (anti-Ro)
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anti-SS-A (anti-Ro):
Sjogren's Syndrome |
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anti-SS-B (anti-La)
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anti-SS-B (anti-La)
Sjogren's Syndrome |
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anti-U1 RNP
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anti-U1 RNP:
Mixed CT Disease |
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anti-smooth muscle Ab
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anti-smooth muscle Ab:
Autoimmune hepatitis |
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anti-glutamate decarboxylase Ab
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anti-glutamate decarboxylase Ab:
Type 1 DM |
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c-ANCA
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c-ANCA:
Wegener's granulomatosis |
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p-ANCA
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p-ANCA:
various vasculitides |
