Flashcards in Immunology Deck (143):
a. What muscles come from first arch?
b. what nerves come from first arch?
a. muscles of mastication, mylohyoid muscle, tensor tympani, tensor veli palatini
b. maxillary and mandibular division of trigeminal nerve
What amino acids are found in elastin?
Glycine and proline
What is mechanism of adenosine as anti-arrhythmic?
Increases outward K current
Hyper polarization of cells
Decreases intracellular Ca
What part of the body drains to:
a. Axillary lymph node
b. Superificial inguinal nodes
c. Popliteal nodes
a. Upper limb, breast, skin above umbilicus
b. anal canal (below pectinate line), skin below umbilicus, scrotum
c. Dorsolateral foot, posterior calf
Which part of the GI tract drains to:
a. Celiac lymph nodes
b. Superior mesenteric nodes
c. Inferior mesenteric nodes
d. Internal iliac nodes
a. liver, stomach, spleen, pancreas, upper duodenum
b. lower duodenum, jejunum, ileum, colon to splenic flexure
c. colon from splenic flexure to upper rectum
d. lower rectum to anal canal (above pectinate line), bladder, vagina, prostate
Which lymph nodes do the testes drain to? the scrotum?
Testes --> PARA-AORTIC
Scrotum --> superficial inguinal
What part of the body does the right lymphatic duct drain?
Right side of body above the diaphragm
What part of the body does the thoracic duct drain?
Everything else besides right side of body above diaphragm. Goes into junction of left subclavian and internal jugular veins.
Where do you find peer's patches?
In lamina propria submucosa of ileum
a. What muscles are derived from second branchial arch?
b. nerves from second branchial arch?
a. Muscles of facial expression, stapedius, stylohyoid
b. Cranial nerve seven
Which cells express MHCI?
ALL cells EXCEPT RBCs
a. What genes code for MHC I?
b. What genes code for MHC II?
a. HLA-A, B and C
b. HLA-DR, DP, and DQ
What diseases does HLA-B27 predispose to?
Inflammatory Bowel Disease
What diseases do HLADR3/4 predispose to?
Diabetes mellitus type I
DR4 - Rheumatoid arthritis
a. What is Langerhans Cell Histiocytosis?
b. What markers does it express?
c. ON electron microscopy?
d. How does it present?
a. Group of proliferative disorders of dendritic cells
b. Express S-100 and CD1a
c. Birbeck granules
d. Presents as child with lytic bone lesion and skin rash or recurrent otitis media with mass involving mastoid bone
What 4 things are expressed on dendritic cells?
What mediates the following reactions:
a. hyperacute transplant rejection
b. acute transplant rejection
c. chronic rejection
d. graft vs. host
a. pre-existing recipient antibodies
b. T cells
c. T cells and Antibodies; Cytotoxic T cells treat transplant cells as self cells presenting non-self antigen - irreversible
d. Usually in BM transplants; the grafted immunocompetent T cells proliferate in the immunocompromised host and reject host cells with "foreign proteins"
How does graft vs. host disease present?
Maculopapular rash, jaundice
c. Adverse effects (unique one?)
a. Binds to cyclophilin --> inhibits Calcineurin --> prevents production of IL2 --> disrupts T cell activation
b. Prevent transplant rejection, psoriasis, RA
c. Viral infections, lymphomas, NEPHROTOXICITY, HTN
c. Adverse effects
a. Bind FK506 binding protein (FKBP) --> inhibits Calcineurin --> prevents production of IL2 --> blocks T cell activation
b. Transplant rejection prophylaxis
c. Increased risk of diabetes and neurotoxicity,, nephrotoxicity, HTN
c. Adverse effects
a. binds FKBP --> inhibits mTOR --> inhibition of T cell proliferation and prevents response to IL2
b. Kidney transplant
c. Not that important - NOT NEPHROTOXIC; anemia, thrombocytopenia, leukopenia
c. Adverse effects
(precursor to 6-Mercaptopurine)
a. Inhibits lymphocyte proliferation by blocking nucleotide synthesis
b. Transplant rejection prophylaxis (especially kidney), RA, glomerulonephritis
c. BONE MARROW suppression; degraded by Xanthine Oxidase (toxic effects can be increased by Allopurinol)
a. Inhibits IMP dehydrogenase --> prevents synthesis of guanine --> no cell replication/proliferation of B cells and T cells
b. Transplant patients and Lupus nephritis
a. Binds CD3 (T cells) --> interferes with T cell signal transduction --> shuts down T cells
a. Binds to CD25 (IL2 receptor on activated T cells) --> blocks IL-2 signaling (Sirolimus is similar)
a. affects TNFa
c. NOT USED - phocomelia, teratogen
a. What are the anti-TNFa agents?
b. What are they used for?
c. Which anti-TNF alpha drug is not a monoclonal antibody?
a. Adalimumab, Infliximab
b. Ankylosing spondylitis, RA, IBD, reactive arthritis
c. Etanercept - mimics TNFa receptor (gobbles up TNFa in circulation)
a. Targets platelet glycoproteins IIb/IIIa
b. Anti-platelet agent for prevention of ischemic complications in patients undergoing percutaneous coronary intervention
a. Ab against HER2
b. Breast cancer that expresses HER2
a. Targets CD20
b. B cell NON-Hodgkin lymphoma
a. Targets IgE
b. Severe asthma
a. defect in BTK (tyrosine kinase gene) --> no B cell maturation; X linked recessive
b. Recurrent bacterial infections and enteroviral infections after 6 months (decreased maternal IgG)
c. Absent B cells in peripheral blood, decrease IgG of ALL classes; absent lymph nodes/tonsils
Selective Ig Deficiency
a. Most common primary immunodeficiency; unknown
b. Majority asymptomatic, can see airway and GI infections; autoimmune disease (atopy, asthma) anaphylaxis to IgA products
c. Decreased IgA with normal IgG and IgM
Common Variable Immunodeficiency
a. Defect in B cell differentiation
b. Can be acquired in 20-30s, increased risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections
c. decreased plasma cells, decreased immunoglobulins
Thymic aplasia (DiGeorge)
a. 22q11 deletion; failure to develop 3rd and 4th pharyngeal pouches --> absent parathyroids and thymus
b. Cardiac abnormality, Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia - recurrent viral, fungal and protozoal infections
c. Decreased T cells, PTH and Calcium; absent thyme shadow on CXR
IL-12 receptor deficiency
a. decreased TH1 response; AR
b. disseminated MYCOBACTERIAL and fungal infections;
c. Decreased IFNy
Chronic Mucocutaneous Candidiases
a. T cell dysfunction; many causes
b. non-invasive Candida albicans infections of skin and mucous membranes
c. absent cutaneous reaction and T cell proliferation in response to Candida antigens
Hyper IgM Syndrome
a. defective CD40L on Th cells --> defect in class switching; X linked recessive (3 types)
b. Severe pyogenic infections early in life; opportunistic infection with Penumocystis, Crypto, CMV
c. Increased IgM, decreased other antibodies
a. defect in early stem cell differentiation; caused by many including adenosine deaminase deficiency (X linked)
b. failure to thrive, chronic diarrhea, thrush, recurrent viral/bacterial/fungal/protozoal infections (treat with BM transplant)
c. Decreased T cell receptor excision circles (TRECs); absence of thymic shadow, germinal centers, and T cells
a. Mutation in WAS gene (X linked) --> T cells unable to recognize actin cytoskeleton
b. Thrombocytopenia purpura, Eczema (on trunk), Recurrent infections
c. Decreased to normal IgG, IgM; Increased IgE and IgA; fewer/smaller platelets
What are the X linked immunodeficiencies?
Chronic granulomatous disease
a. defects in ATM gene --> failure to repair DNA double strand breaks --> cell cycle arrest
b. Triad = Cerebellar defects (ATAXIA), spider Angiomas (telangiectasia) IgA deficiency
c. Increased AFP, decreased IgA, IgG an IgE; lymphopenia, cerebellar atrophy
Chronic Granulomatous Disease
a. defect in NADPH oxidase --> decreased reactive oxygen species and respiratory burst in neutrophils
b. Increased susceptibility to catalase + organisms (Nocardia, Pseudomonas, Listeria, Aspergillus, Candida, E. coli, S. aureus, Serratia
c. Abnormal dihydroorhodamine test; nitroblue tetrazolium dye reduction test is negative
How is CGD treated?
a. defective LYST gene --> defective lysosomal transport --> microtubule dysfunction in pharosome-lysosome fusion (AR)
b. Recurrent pyogenic infections by Staph and Strep; partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistiocytosis
c. giant granules in granulocytes and platelets, pancytopenia, coagulation defects
Leukocyte adhesion deficiency type 1
a. defect in LFA-1 integrin (CD18) on phagocytes, impaired migration and chemotaxis (AR)
b. recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing, delayed separation of umbilical cord
c. Increased neutrophils, absence of neutrophils at infection sites
What cytokines do T helper cells produce to stimulate B cells?
What are the surface markers of B cells?
CD19, CD20, CD21, IgM, IgD
What cell markers do T cells have?
CD3, CD28, T cell receptor
Cytotoxic T cells have CD8
Helper T cells have CD4 and CD40L
What cell markers do NK cells have?
CD56 and CD16
What cell markers do macrophages have?
CD14, MHC II, B7 protein, CD40
Which organism's exotoxin works via each of the following mechanisms?
a. inactivates EF2
b. activates Gs
c. Disables Gi
d. Bacterial adenylat cyclase (No G protein involved)
e. Blocks GABA and glycine
a. Corynebacterium diptheriae, Pseudomonas (exotoxin A)
b. Vibrio cholerae, E. coli (ETEC heat labile toxin)
c. Pertussis toxin
d. Bacillus anthracis
e. Clostridium tetani (lockjaw)
What drug helps to make diagnosis of Myasthenia gravis?
Edrophonium (acetylcholinesterase inhibitor)
Too short acting to treat
What heart sound is associated with dilated CHF?
What heart sound is associated with stiffened LV?
What is amyloidosis?
Accumulation of one specific protein in the tissues that aggregates into B pleated sheets which causes damage and apoptosis
What is AL amyloidosis? What protein is deposited? Which organs does it affect?
Due to deposition of proteins from Ig Light chains
Can occurs as plasma cell disorder or associated with multiple myeloma.
Often affects multiple organ systems including renal (nephrotic syndrome), cardiac (restrictive CM, arrhythmia), hematologic (brushing, splenomegaly), GI (hepatomegaly) and neurologic (neuropathy)
What is AA amyloidosis? What protein is deposited? What is it associated with?
Seen in chronic inflammatory conditions such as RA, IBD, spondyloarthropathy
Fibrils composed of serum Amyloid A; often multi system
a. Patient with fibrils made of B2 microglobulin, ESRD and/or long term dialysis.
b. What else may it present as?
a. Dialysis-related amyloidosis
b. Carpal tunnel syndrome
Which protein and fibril with each form of amyloidosis?
a. type 2 diabetes
b. Medullary thyroid CA
d. Dialysis -associated
a. amylin, AIAPP
b. Calcitonin, A-CAL
c. B-amyloid, APP
d. B2 microglobulin, AB2M
How do you diagnosis amyloidosis on biopsy?
Congo red stain; apple green birefringence
Disease associated with anti-IgG antibodies
Disease associated with anti-citrullinated protein antibodies (ACPA)
Associated with anti-centromere ABs
(C for centromere, C for CREST)
Associated with Anti-Scl-70 Abs
(Scl = Scleroderma)
Associated with Anti-histone Abs
Associated with anti-nuclear Abs (ANA)
Associated with Anti-smith Abs
Associated with Anti-dsDNA Abs
Associated with Anti-Jo-1 Abs
Associated with Anti-SSA (anti-Ro)
Associated with Anti-SSB (anti-La)
Associated with Anti-U1-RNP Abs
Mixed CT disease
Associated with Anti-desmoglein Abs
Associated with Anti-acetylcholine receptor Abs
Associated with Anti-endomysial (anti-tissue transglutaminase)
Associated with Anti-gliadin Abs
Associated with anti-mitochondrial Abs
Primary Biliary Cirrhosis
Associated with Anti-smooth muscle Abs
Associated with anti-glutamate decarboxylase Abs
Type 1 Diabetes
Associated with Anti-thyrotropin receptor Abs
Associated with Anti-thyroid peroxidase (anti-TPO)
Hashimoto thyroiditis, Graves disease
Anti-Basement membrane Abs
Granulomatosis with polyangiitis (Wegener's)
Pauci-immune crescentic glomerulonephritis
Which vaccines are dangerous for patients with egg allergy?
What mediates Type I Hypersensitivity?
Free antigen cross-linkes IgE on presensitized mast cells and basophils --> triggers immediate release of vasoactive amines that act on post capillary venules (histamine and bradykinin)
What mediates Type II Hypersensitivity?
Cytotoxic (Ab mediated); IgM an IgG bind to FIXED antigen on "enemy" cell and cause cellular destruction by opsonization/phagocytosis, complement mediated inflammation or Ab-mediated cellular dysfunction
What is Serum sickness?
Type III hypersensitivity reaction where antibodies to foreign proteins are produced --> immune complexes form and are deposited in membranes where they fix complement (cause tissue damage); mostly caused by drugs; happens 5-10 days after antigen exposure
What are signs of serum sickness?
Fever, urticaria, arthralgia, proteinuria, lymphadenopathy
What is Arthus reaction?
Local subacute antibody mediated type III hypersensitivity reaction caused by intradermal injection of antigen; takes 4-12 hours
What are signs of Arthus reaction?
Edema, necrosis, activation of complement
Acute hemolytic transfusion reaction
Acute rheumatic fever
Bee sting allergy
Guillain Barre syndrome
Seasonal allergic rhinitis
What is the starting point for the Classic complement pathway?
IgG or IgM recognize C1
What is C3b important for?
What are the two most important opsonins?
C3b and IgG
What are C3a and C5a important for?
C3a - stimulates mast cells and basophils
C5a - neutrophil chemotaxis
a. What happens if you are deficient in C1 esterase inhibitor?
b. What drug is C/I?
a. Hereditary angioedema (from elevated Bradykinin)
b. ACE inhibitors are C/I
What happens if you are deficient in C3?
Recurrent pyogenic sinus and respiratory infections (Strep pneumo and H influenzae)
Also increased susceptibility to type III hypersensitivity and pyelonephritis
What happens if you are deficient in C5b-C9 proteins?
Susceptible to recurrent Neisseria bacteremia
a. What is DAF?
b. What happens if you have DAF deficiency?
a. GPI anchored enzyme (CD55)
b. Causes complement mediated lysis of RBCs and Paroxysmal Nocturnal Hemoglobinuria
What kinds of organisms are asplenic patients susceptible to?
Even Some Pretty Nasty Killers Have Shiny Bodies
Group B Streptococcus
Why would patient be missing spleen?
Sickle cell patient
Hereditary spherocytosis - RBCs are defective and spleen keeps removing them
What are macrophages activated by? What cells produce it?
Produced by TH1 and NK cells
Which cytokines do macrophages make?
What does IL-12 do?
Induces differentiation of T cells into TH1 cells
Activates NK cells
What are the neutrophil chemotactic factors?
IL8, C5a, leukotriene B4
What is Aldesleukin? What is it used for?
Recombinant IL-2 used for renal cell carcinoma and metastatic melanoma
What are Filgrastim and Sargramostim? What are they used for?
Recombinant GM-CSF used for recovery of bone marrow
What is Oprelvekin?
Recombinant IL-11 used for thromobocytopenia
What is recombinant IFN-a used for?
Hepatitis B/C, Kaposi sarcoma, malignant melanoma, genital warts, hairy cell leukemia
What is recombinant IFN-b used for?
What is recombinant IFN-y used for
Chronic Granulomatous Disease
What cytokines do TH1 cells produce?
IL-2 and IFNy
What cytokine inhibits macrophage activation?
What does IL-10 inhibit?
Production of TH1 cells and macrophage activation
What inhibits production of TH2 cells?
What muscles and nerves are derived from third branchial arch?
Glossopharyngeal nerve (IX)
What kind of drug is used in treatment of Myasthenia gravis?
Acetylcholinesterase inhibitors like Pyridostigmine, Physostigmine
What cytokines enhance NK cell activity?
What do patients with common variable immunodeficiency have increased risk of later in life?
Autoimmune disease and lymphoma