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69 Cards in this Set
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– Recurrent bacterial infections after 6 mo.
– All Ig's decreased-> Opsonization dfx |
Bruton's agammaglobulinemia
–defective tyrosine kinase –X-linked recessive –immature B cells |
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– Severe pyogenic infections EARLY in life
– Increased IgM – Very low IgE, IgA, & IgG |
Hyper-IgM Syndrome
–Defective Th2 CD40 Ligand-> –Inability to class switch (^IgM^) –Often X-linked |
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–Anaphylaxis upon exposure to IgA (blood products)
–Often sinus/lung infections, milk allergy, diarrhea |
Selective Ig (A!) Deficiency
–Defective isotype switching in a specific class – IgA by far most common – Show low levels of secretory IgA |
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– Young Adults (20s & 30s)
– Normal # B-cells – Decreased # Plasma cells – Decreased Ig levels (duh) – Co-morbid AI Dx |
Common Variable Immunodeficiency (CVID)
–B-cell maturation defect (multi-factoral) –Often co--morbid AI dx • Rh Arthritis • Addison Dx • Thyr |
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– Congenital+Great Vessel dfx
– Recurrent viral/fungal infec. – Absent cardiac shadow – Hypocalcemia (+tetany +Trousseau/Chvostek) |
Velocardialfacial Syndrome (thymic aplasia)
–Deletion of 22q11 – Failure of 3rd and 4th pharyngeal pouch development – No Tcells (thymus) and No PTH (parathyroid) |
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–"Cold" (non-inflammatory) Abcesses, usually staph.
–Eczema –Retained baby teeth –Coarse facies+frontal bossing –Increased IgM levels |
Hyper-IgE Syndrome OR
Job Syndrome – Th1 cells fail to make IFN-g – Inability for PMNs to respond to chemtoxis stim. |
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C ardiac abnormality
A bnormal face T hymic aplasia C left palate H ypocalcemia 22 chr. deletion |
CATCH 22 = Velocardiofacial Syndrome = DiGeorge Syndrome
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–Recurrent viral, fungal, bacterial infections
–Absent thymic shadow –No germinal centers on lymph node biopsy –No Bcells on blood smear – Only cure is bone marrow transplant |
Severe Combined Immunodeficiency (SCID)
–X-linked adenosine deaminase deficiency (adenine is toxic to B/T cells) –Failure to synthesize Ag's onto MHC II –Many types, defective IL-2 receptor most common |
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–Recurrent sinusitis (peds)
–Cerebellar dfx –Poor smooth pursuit –IgA deficiency –Telangiectasia (spider angiomata) |
Ataxia-Telangiectasia
–DNA repair enz dfx (ATM gene) –CT/X-rays are CONTRAINDICATED for both dx pts AND gene carriers! PEARL: Don't wait for telangiectasias; pay attn for smooth pursuit dfx (they are first!) |
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–Recurrent infections
–Eczema –Thrombocytopenic purpura –Incr/Norm IgE/IgA – Low levels IgM (weird!) |
Wiskott-Aldrich Syndrome
–Progresssive deletion of B/Tcells –WASP gene dfx –X-linked recessive |
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Three most important X-linked Immunodeficiencies?
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WBS
W iskott-Aldrich B rutons agammaglobulinemia S evere Combined Imm. Def BONUS: What other Imm Def is X-linked 70% of the time? (answer=hyperIgMsyndrome) |
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Bee Sting/Food Allergy
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Type I
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Atopic dxs
–Hay fever –Eczema –Asthma |
Type I
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Hemolytic Anemia
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Type II
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Pernicious Anemia
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Type II
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ITP
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Type II
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Erhythroblastosis fetalis ("HDN")
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Type II
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Acute hemolytic transfusion rxn
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Type II
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Rheumatic fever
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Type II
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Bullous pemphigoid
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Type II
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Pemphigous vulgaris
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Type II
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Graves dx
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Type II
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Myasthenia Gravis
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Type II
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SLE
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Type III
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Rh Arthritis
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Type III
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Polyarteritis nodosum (PAN)
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Type III
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Post Strep GLN
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Type III
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Serum sickness
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Type III
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Arthus rxn (e.g. swelling/inflamm post tetnus vax)
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Type III
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Farmer's Lung (aka Hypersensitivity pneumonitis)
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Type III
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Type 1 DM
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Type IV
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Multiple Sclerosis (MS)
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Type IV
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Prior exposed T-cells encounter Ag's and release cytokines (Mø's then activate)
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Type IV
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Guillian-Barre
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Type IV
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Hashimoto Thyroiditis
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Type IV
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Graft versus host dx (GVH)
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Type IV
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PPD sin test for TB
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Type IV
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Contact dermatitis
• Urushiol • Nickel • Latex |
Type IV
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Prior exposed T-cells encounter Ag's and release cytokines (Mø's then activate)
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Type IV
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Anti-bodies bind to host cells
Complement activated (MAC) Mø's & PMN cause dmg |
Type II
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Ag-Ab complexes deposit & activate complement
PMNs release lysosomal enz. |
Type III
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Free Ag cross links IgE on PRE-SENSITIZED Mast cells/Basophils
What substance is released? Where? |
Type I rxn
Histamine released @Post-capillary venule |
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FOUR important LIVE (attenuated) vaccines:
1.) 2.) 3.) 4.) |
1.) MMR
2.) Polio (oral; Sabin) 3.) VZV (chix pox vax) 4.) Yellow Fever |
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What is one + and one – to a LIVE (attenuated) vaccine?
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+ Lifelong immunity
– Can revert to virulent form if immuno comprimised |
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FIVE important KILLED (inactivated) vacines:
1.) 2.) 3.) 4.) 5.) |
CHIRP
1.) Cholera 2.) Hepatitis A 3.) Influenza 4.) Rabies 5.) Polio (injection; Salk) |
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Vaccine type that induces a CELLUAR (CD8) response?
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LIVE vax
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Vaccine type that grants HUMORAL immunity
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KILLED vax
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What is one + and one – to KILLED (inactivated) vaccine?
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+ Safer and more stable
– Weaker imm resp: boosters required |
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IFN-a and IFN-b inhibit viral ptn synthesis by DEGRADING viral mRNA
How do they do this? |
IFNa/IFNb induce the production of ribonuclease that will cleave viral mRNA but NOT host mRNA (cool huh?)
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Unique cytokine marker for NK cells?
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CD 56
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What drug(s) fit this description?
–similar to cyclosporine –SIDE FX: Nephrotoxicity Pleural Effusion Neuropathy HTN |
Tacrolimus
Pimicrolimus |
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What drug(s) fit this description?
–binds to FK-binding ptn; blocks IL-2 secretion |
Tacrolimus
Pimicrolimus |
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What drug(s) fit this description?
–calcineurin inhibitor; blocks IL-2 production |
Cyclosporine
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What drug(s) fit this description?
–binds cyclophilins –SIDE FX: Gout Nephrotoxicity (prevented w/mannitol) Increased viral infections, lymphoma |
Cyclosporine
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What drug(s) fit this description?
–binds to FK-binding ptn; inhibits mTOR causing decreased T-cell response to IL-2 (decreased proliferation) |
Sirolimus (rapamycin)
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What drug(s) fit this description?
–monoclonal AB that blocks IL-2 receptors (receptors located on activated T-cells) |
Daclizumab
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What drug(s) fit this description?
–blocks nucleic acid synthesis –toxic to proliferating lymphocytes –Upstream cousin of 6-MP (precursor) |
Azathiaprine
(allopurinol contraindicated, why?) |
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What drug(s) fit this description?
–monoclonal AB to CD3 marker (epsilon chain) (CD3 is responsible for all T-cell signal transduction) |
Muromonab-CD3 (OKT3)
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What drug(s) fit this description?
–Anti-TNF-alpha AB 1.)_____________ 2.)_____________ |
1.) Infliximab
2.) Adalimumab |
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What drug(s) fit this description?
–Anti-CD20 AB –used in B-cell Hodgkin dx |
Rituximab
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What drug(s) fit this description?
–Anti-IIb/IIIa glycoprotein AB –used for unstable angina |
Abcixumab
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What drug(s) fit this description?
–Anti-erb-B2 AB –used on +HER2 breast cancers |
Trastuzumab (HERCEPTIN)
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What drug(s) fit this description?
–Anti-IgE AB –used for severe refractory asthma |
Omalizumab
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Name 6 diseases treated with Anti-TNF-a ABs:
1.) ________________ 2.) Rhematoid Arthritis 3.) P______________ 4.) A______________ 5.) I_______________ 6. R_______________ |
1.) Crohn Dx
2.) Rheumatoid Arthritis 3.) Psoriatic Arthritis 4.) Ankylosing Spondylitis 5.) IBD 6.) Reiter Syndrome |
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What disease(s) is IFN-alpha useful for?
1.) Hep B 2.) ____________ 3.) ____________ 4.) ____________ 5.) ____________ |
Hep B
Hep C Kaposi Sarcoma Leukemias Malignant Melanoma |
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What disease is IFN-ß useful for?
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Multiple sclerosis
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What disease is IGN-g useful for?
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Chronic Granulomatous Dx
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What drug(s) fit this description?
–inhibits Inosine Monophosphate Dehydrogenase –Prevents production of GUANINE |
Myocphenolate
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What drug(s) fit this description?
–Anti Angiogenesis properties –Useful in Multiple myeloma (separate mechanism) |
Thalidomate
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