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step1 Immun FC-RX

Immunology- didnt know

The hinge region of an antibody connects which 2 domains? The Fab region of the heavy chain and the Fc region; specifically, CH1 and CH2 domains
What are the 4 domains that comprise the Fab region of an antibody? Variable light region (VL), constant light region (CL), variable heavy region (VH), constant heavy 1 region (CH1)
Which antibody isotype acts as the main antibody in the secondary, delayed response to an antigen? IgG
List 4 main functions of IgG. 1. Fixation of complement, 2. opsonization of bacteria, 3. neutralization of bacterial toxins and viruses, 4. provision of passive immunity to infants via placenta
Which cell surface proteins are found on macrophages? CD14, CD40, CCR5, MHC II, B7 (CD80/86), and receptors for Fc and C3b
IgA crosses epithelial cells by transcytosis. In which structural form is IgA secreted? Dimeric form, with J chain (monomeric form only found in serum)
Unlike other immunoglobulin (Ig) isotypes, the function of IgD is unclear. Where can immunoglobulin D be found? Surface of many B cells and in serum
To what specific region(s) of the heavy chain do macrophages bind? CH2 and CH3 regions
Which molecular cofactor is required for both the creation and neutralization of reactive oxygen species (ROS)? NADPH
Which type of influenza vaccine is theoretically safer for most patients to receive? The injected influenza vaccine, which is killed/inactivated (unlike the intranasal influenza vaccine, which is live attenuated)
What 3 symptoms are seen in patients with Wiskott-Aldrich syndrome? Wiskott-Aldrich: Thrombocytopenia, Eczema, Recurrent (pyogenic) infections (WATER)
DiGeorge syndrome, IL-12 receptor deficiency, Job syndrome, and chronic mucocutaneous candidiasis feature dysfunction of which cell type? T cells
What is the pathophysiology of leukocyte adhesion deficiency type 1? Lack of LFA-1 integrin (CD18) on phagocytes → impaired migration and chemotaxis
Anti-U1 RNP (ribonucleoprotein) autoantibodies are associated with which disorder? Mixed connective tissue disease
A patient with leukemia receives a bone marrow transplant and develops graft-versus-host disease. How is this a potentially desirable effect? This may be beneficial in the eradication of leukemia cells (the "graft-versus-tumor" effect)
A 6-year-old girl with nausea, vomiting, polyuria, and sudden weight loss has a blood glucose level of 800 mg/dL. Lab tests for which autoantibodies should be ordered? Anti-glutamic acid decarboxylase and islet cell cytoplasmic autoantibodies (to check for type 1 diabetes)
Which type of vaccine stimulates the immune system to make antibodies without potential for causing disease? Toxoids
Bruton agammaglobulinemia, selective IgA deficiency, and common variable immunodeficiency are disorders of which type of leukocyte? B cells
What general type of vaccine includes only the antigens that best elicit an immune response? Subunit vaccines
Which immunodeficiency results in low T-cell counts, ↓PTH, and ↓Ca2+? Thymic aplasia=both erd and 4thPP (which can lead to tetany secondary to parathyroid aplasia, recurrent viral/fungal infection due to ↓T cells). note pth thymus and inferior parathyroid derived from third pharyngeal pouch(PP), superior paratyroid from 4
Which immunodeficiency syndrome is the most common cause of Mendelian susceptibility to mycobacterial diseases (MSMD)? IL-12 receptor deficiency
Rheumatoid factor, often positive in rheumatoid arthritis, is an autoantibody targeting which immunoglobulin? It is an IgM antibody against the Fc region of IgG
What can be observed on flow cytometry and lymph node biopsy from a patient with severe combined immunodeficiency? Lack of T cells on flow cytometry and no germinal centers in lymph nodes
A patient presents with swelling and pain in her PIP and MCP joints, bilaterally. Of the autoantibodies associated with this condition, which are more specific? Anti-CCP autoantibodies (this is rheumatoid arthritis)
MPO-ANCA/p-ANCA autoantibodies are associated with which 3 disorders? Microscopic polyangiitis, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), and ulcerative colitis
What gene mutation leads to autosomal dominant hyper-IgE syndrome (Job syndrome)? STAT3 mutation, which leads to Th17 deficiency, resulting in impaired neutrophil recruitment to infection sites
What does a live attenuated vaccine contain? A microorganism that has been modified to lose its pathogenicity (though it retains the capacity for transient growth within the host)
A 4-year-old girl is diagnosed with common variable immunodeficiency. What type of poliovirus vaccine may be contraindicated? The Sabin oral vaccine (live, attenuated polio vaccines are contraindicated for patients with B-cell deficiencies)
hich 2 types of hypersensitivity reactions are implicated in the development of chronic transplant rejection? Type II and IV hypersensivity (involving both humoral and cellular components)
A kidney transplant recipient experiences gradual oliguria. Biopsy reveals vasculitis of graft vessels with a lymphocytic cellular infiltrate. What type of rejection is present? Acute rejection
What is the pathogenesis of chronic granulomatous disease? Defective NADPH oxidase → ↓ ROS and oxidative burst → ↑ susceptibility to catalase ⊕ organisms
Myasthenia gravis, Graves disease, and pemphigus vulgaris are examples of what type II hypersensitivity mechanism? Cellular dysfunction, in which the opsonized cells do not function normally but are not completely destroyed
After a camping trip, a patient presents with an itchy, red, oozing rash on the lower extremities, sparing the soles of his feet. What is the diagnosis? Contact dermatitis, likely caused by poison ivy (a type IV hypersensitivity reaction) linear pattern
What test is used to determine T-cell immune function, via a type IV hypersensitivity-mediated reaction? Candida skin test
What are 10 key examples of live vaccines? Adenovirus/Typhoid/Polio/Varicella/ Smallpox/BCG/Yellow fever/Influenza/ MMR/Rotavirus (Attention Teachers! Please Vaccinate Small, Beautiful Young Infants with MMR Regularly)
A man comes to the clinic with a disseminated mycobacterial infection after receiving the BCG vaccine. He has low IFN-γ levels. Which leukocytes are affected in this patient? Th1 cells (he has IL-12 receptor deficiency)
What cardiac defects are typically associated with DiGeorge syndrome? Conotruncal abnormalities, such as tetralogy of Fallot and truncus arterious
What conditions might be seen in patients with common variable immunodeficiency? Autoimmune diseases, bronchiectasis, lymphoma, sinopulmonary infections
What is the classic triad of presentation seen in ataxia telangiectasia? Ataxia, spider Angiomas, decreased IgA
What is the general difference between infections commonly associated with T-cell and B-cell deficiencies? T-cell deficiencies tend to produce recurrent viral/fungal infections, while B-cell deficiencies tend to produce recurrent bacterial infections
What is a specific pathologic manifestation of chronic rejection in a transplanted lung? Bronchiolitis obliterans
What are the characteristic lab findings in Wiskott-Aldrich syndrome? Normal/decreased IgG and IgM, elevated IgE and IgA, thrombocytopenia with microsized platelets
A patient has a history of several miscarriages and venous thromboses. Labs show prolonged PTT. What autoantibodies are associated with this syndrome? Anti-β2 glycoprotein I, lupus anticoagulant, and anticardiolipin autoantibodies (she has antiphospholipid syndrome)
A young child has no B cells in peripheral blood, low immunoglobulins, and scanty tonsils. What is the diagnosis? X-linked (Bruton) agammaglobulinemia
Name the 2 primary autoantibodies associated with Hashimoto thyroiditis. Antithyroglobulin and antithyroid peroxidase (antimicrosomal) autoantibodies
Patients with ataxia-telangiectasia are at increased risk for which malignancies? Lymphomas and leukemias
A child has a fever, headache, flushing, and chills 4 hr after receiving a blood transfusion. How could this be prevented in the future? By using leukoreduced blood (in which WBCs have been removed); this is febrile nonhemolytic reaction (mediated by cytokines released from accumulated donor WBCs)
How is a killed vaccine prepared? The pathogen is inactivated with heat or chemicals before administration (leaving the epitope structure on surface antigens intact)
A new mother expresses concern that her baby will have a "bad reaction" to the Hib (Haemophilus influenzae type b) vaccine. What is the correct response? The chance of an adverse reaction to a subunit vaccine (eg, Hib) is extremely low, even relative to other vaccine types
A child has recurrent bacterial skin infections with absence of pus and impaired wound healing. What lab findings might confirm the diagnosis? Increased serum neutrophil levels but lack of neutrophils at infection sites (this is type 1 leukocyte adhesion deficiency)
Name the autoantibodies associated with dermatomyositis and polymyositis. Antisynthetase (eg, anti-Jo-1), anti-SRP, and anti-helicase (anti-Mi-2)
A woman has ataxia, weblike angiomas, low IgA levels, high AFP, and cerebellar atrophy on imaging. Which gene is defective? The ATM gene: ataxia-telangiectasia is due to ATM gene defects encoding DNA repair enzymes
A mother is Rh- and her fetus may be Rh+. Which Coombs test should each of them receive? Test mother blood for Rh+ antibodies with indirect Coombs test; test fetal blood for antibodies adhered to RBCs with direct Coombs test
A patient with HIV infection has a CD4 count of 600 cells/mm3. What are the 2 live attenuated vaccines that can be given to this patient? MMR and varicella vaccines can be given to HIV+ patients with a CD4 cell count ≥ 200 cells/mm3
A patient with pancytopenia who is undergoing marrow chemoablation in preparation for bone marrow transplantation requires blood products. What must be done before transfusion? The blood products must be irradiated (as this helps prevent GVHD in immunocompromised patients)
What are 6 key examples of subunit vaccines? HBV (antigen is HBsAg), HPV (types 6, 11, 16, 18), acellular pertussis (aP), Neisseria meningitidis, Streptococcus pneumoniae, Haemophilus influenzae type b
What is the pathogenesis of Wiskott-Aldrich syndrome? Mutation in WAS gene leads to inability of leukocytes and platelets to reorganize actin cytoskeleton, resulting in defective antigen presentation
What is serum sickness? An immune complex disease; antibodies to foreign proteins are made and deposited in tissues, where they fix complement and cause damage
What effect can a T-cell deficiency have on a bacterial infection? Increased risk of sepsis (due to rapid dissemination of the infection)
What are the modes of inheritance of leukocyte adhesion deficiency (type 1) and Chédiak-Higashi syndrome? Both have an autosomal recessive inheritance pattern
A patient receiving induction chemotherapy for acute leukemia has severe granulocytopenia. To what types of microbes is she most susceptible? Bacteria (Some Bacteria Produce No Serious granules: Staphylococcus, Burkholderia, Pseudomonas, Nocardia, Serratia) and fungi (systemic Candida, Aspergillus, Mucor)
A patient with fevers and a new heart murmur is found to have tricuspid endocarditis. Blood cultures grow yeasts with pseudohyphae. What immune cell count may be reduced? Granulocytes may be deficient (increasing the risk of systemic or bloodstream candidal infections)
Which condition caused by T-cell dysfunction presents with persistent noninvasive Candida infection? Chronic mucocutaneous candidiasis
An hour after blood transfusion, a patient has a fever, dyspnea, flank pain, and jaundice. Which type of hypersensitivity reaction is this? Type II hypersensitivity reaction, an acute hemolytic transfusion reaction mediated by antibodies to donor RBC surface antigens, with extravascular hemolysis (jaundice)
An hour after blood transfusion, a patient has a fever, dyspnea, flank pain, and jaundice. Which type of hypersensitivity reaction is this? Type II hypersensitivity reaction, an acute hemolytic transfusion reaction mediated by antibodies to donor RBC surface antigens, with extravascular hemolysis (jaundice)
List the symptoms of hyper-IgE (Job) syndrome using the mnemonic ABCDEF Noninflamed staphylococcal Abscesses, retained Baby teeth, Coarse facies, Dermatologic problems (eczema), high IgE, Fractures from minor trauma (Learn the ABCDEF’s to get a Job!)
A patient with IgA deficiency develops wheezing, hypotension, and respiratory arrest after receiving a blood transfusion. What is the most likely cause? Type I hypersensitivity reaction to plasma proteins in donor blood; patients with IgA deficiency must receive blood products that do not contain IgA
What autoantibodies are associated with diffuse scleroderma? Anti-Scl-70 (anti-DNA topoisomerase I [Scleroderma])
What is the pathogenetic mechanism whereby hyperacute rejection develops? Pre-existing recipient antibodies react to donor antigens (type II hypersensitivity), causing thrombosis of graft vessels and necrosis
What is a common histologic manifestation of chronic rejection of a liver transplant? Vanishing bile duct syndrome
Which immunodeficiency can cause a false-positive β-hCG test result? Selective IgA deficiency (most common primary immunodeficiency; unknown cause)
A young patient is found to have an absence of B cells. To which types of viruses is he most susceptible? Enteroviruses, including poliovirus (especially manifesting as enteroviral encephalitis)
The umbilical cord fails to separate within 30 days in a neonate. Neutrophilia is noted on labs. What is the diagnosis? Leukocyte adhesion deficiency type 1
What is the main drawback associated with toxoid vaccines? They stimulate the production of antitoxin levels that decrease with time (requiring subsequent boosters)
Which 4 types of HPV does the standard quadrivalent subunit HPV vaccine protect against? Types 6, 11, 16, and 18
A patient develops hives, fever, and shock within minutes of receiving a blood transfusion. What is the pathogenesis behind this transfusion reaction? Type I hypersensitivity reaction against plasma proteins in the transfused blood (this is an allergic/anaphylactic reaction)
What are the common causes of serum sickness-like reactions? Drugs such as penicillin (acting as haptens) and infections such as hepatitis B
Which vaccine against Salmonella typhi poses a lower risk of causing infection? The intramuscular Vi polysaccharide typhoid vaccine, which is killed/inactivated
What antibodies are associated with autoimmune hepatitis type 1? Anti-smooth muscle autoantibodies
What 3 immunoglobulin isotypes are typically decreased in patients with ataxia-telangiectasia? IgA, IgG, and IgE
What is the pathogenesis of a delayed hemolytic transfusion reaction? An anamnestic response to a foreign antigen (usually non-ABO antigens previously encountered by the recipient) on the donor RBCs
What is the most common cause of hyper-IgM syndrome? Defective CD40L on T-helper (Th) cells, which is necessary for B-cell class switching
When a cell is opsonized (coated) by antibodies in a type II hypersensitivity reaction, what are the cell's 2 potential fates? Phagocytosis and/or complement system activation or destruction by natural killer cells (antibody-dependent cellular cytotoxicity)
A patient develops respiratory distress due to noncardiogenic pulmonary edema within 4 hours of receiving blood products. What is the cause? Transfusion-related acute lung injury
Describe the mechanism of serum sickness. Antibodies to foreign proteins are produced; 1–2 wk later, antibody-antigen complexes are formed and deposited in tissue → complement activation → inflammation/tissue damage
A 30-year-old woman has exophthalmos, palpitations, diarrhea, and thyromegaly. What autoantibodies cause this condition? Anti–thyroid-stimulating hormone receptor autoantibodies (she has Graves disease)
Autoimmune hemolytic anemia, ITP, transfusion reaction, and hemolytic disease of the newborn are examples of which mechanism of type II hypersensitivity? Cellular destruction in which cells are opsonized and then undergo either NK cell killing or phagocytosis and/or complement system activation
A previously healthy woman, not taking any medications, develops dry eyes and dry mouth. What autoantibodies can cause this? Anti-Ro/SSA and anti-La/SSB autoantibodies, often positive in Sjögren syndrome
A 52-year-old man, diagnosed with small cell lung cancer, has muscle weakness that gets better throughout the day. Which autoantibodies are associated with this disorder? Anti-presynaptic voltage-gated calcium channels; he has Lambert-Eaton myasthenic syndrome, a paraneoplastic syndrome associated with small cell lung cancer
A boy has coarse facies, retained baby teeth, and recurrent abscesses. Labs show ↑ IgE and ↑ eosinophils. Which immune cells are impaired? Th17 cells (he has hyper-IgE syndrome [Job syndrome])
Although most individuals with IgA deficiency have no symptoms, what symptoms can be present that allow for diagnosis? Airway and GI infections, Autoimmune disease, Atopy, and Anaphylaxis to IgA-containing products
What pathogenetic mechanism drives the development of graft-versus-host disease? Grafted immunocompetent T cells proliferate in the immunocompromised host and reject cells with foreign proteins, causing severe organ dysfunction
What live viral vaccine is nonattenuated and only given to military recruits? Adenovirus
A patient develops worsening edema and necrosis at the site of recent tetanus vaccination. What is the diagnosis? Arthus reaction: preformed antibodies attack injected antigen leading to formation of immune complexes in the skin and complement activation
Name the 3 autoantibodies associated with celiac disease. IgA and IgG deamidated gliadin peptide, IgA anti-endomysial, IgA anti-tissue transglutaminase autoantibodies
A boy has thrombocytopenia, eczema, and recurrent pyogenic infections. This patient has an increased risk for what conditions? Malignancy and autoimmune diseases (he has Wiskott-Aldrich syndrome)
What is the mechanism of injury in transfusion-related acute lung injury? Sequestered neutrophils: 1) are primed in pulmonary vessels; 2) get activated in transfused blood and release inflammatory mediators → ↑capillary permeability → pulmonary edema
What is the pathogenetic mechanism whereby acute cellular rejection develops? CD8+ T cells and/or CD4+ T cells are activated against foreign (donor) MHCs in a type IV hypersensitivity reaction
How rapidly does acute (cellular or humoral) transplant rejection develop? Within weeks to months of transplantation
Name 4 inherited immunodeficiencies that are X-linked recessive and thus are more common in boys. Hyper-IgM syndrome, Wiskott-Aldrich syndrome, chronic granulomatous disease, Bruton agammaglobulinemia (and some types of severe combined immunodeficiency)
Which disease is associated with anti-hemidesmosome autoantibodies? Bullous pemphigoid
What are 5 key examples of inactivated or killed vaccines? Hepatitis A, Typhoid (Vi polysaccharide, IM), Rabies, Influenza (injected), Polio (SalK = Killed) (A TRIP could Kill you)
A child with an inherited immune disorder has greasy, foul-smelling stools. What type of immunodeficiency is likely? B-cell deficiency with no IgA (giardiasis), leading to lack of mucosal immunity
A patient experiences severe graft dysfunction within minutes of kidney transplantation. What is the correct treatment? Removal of the graft (the appropriate treatment for a patient with hyperacute rejection, which occurs within minutes of transplantation)
Which disease is associated with antimitochondrial autoantibodies? Primary biliary cholangitis
How does the humoral type of acute rejection differ from hyperacute rejection in its pathophysiology? Antibodies against graft donor antigen(s) develop after transplantation in acute rejection (vs pre-existing antibodies in hyperacute rejection)
Describe the difference between a direct and an indirect Coombs test. Direct: detects antibodies that are already bound to RBCs; indirect: detects free serum antibodies that can bind to RBCs
Which immunodeficiency is due to a defect in B-cell differentiation resulting in decreased plasma cell levels? Common variable immunodeficiency (CVID)
Goodpasture syndrome, rheumatic fever, and hyperacute transplant rejection exemplify what type II hypersensitivity mechanism? Inflammation, in which cell opsonization leads to complement system activation and Fc receptor–mediated inflammation
What is a specific pathologic manifestation of chronic rejection in a transplanted heart? Accelerated atherosclerosis
What Ig isotype most commonly forms the immune complexes implicated in type III hypersensitivity reactions? IgG
A patient has recurrent infections with encapsulated organisms, and B-cell counts are normal. What other immune disease may the patient have? Early complement deficiency
What is the mechanism of the hypersensitivity reaction involved in polyarteritis nodosa? In type III hypersensitivity, antigen-antibody complexes activate complement, leading to neutrophil chemotaxis and immune complex deposition
A pale, blond patient has recurrent pyogenic infections, lymphohistiocytosis, and neuropathy. Pancytopenia is noted. What cellular process is affected in this condition? Defect in lysosomal trafficking regulator gene LYST leads to microtubule dysfunction, impairing phagosome-lysosome fusion (this is Chédiak-Higashi syndrome)
A patient agrees to receive the annual injected influenza vaccine. What is the main drawback of this type of vaccination? Because it is an inactivated vaccine, the immune response is weaker (booster shots or repeated vaccinations are often required)
A patient has a rash on her cheeks, arthralgias, and antibodies to ANA and dsDNA. What type of hypersensitivity reaction causes this disease? Type III (the patient has SLE)
Describe the mechanism of the late phase of a type I hypersensitivity reaction fter mast cell degranulation, chemokines attract inflammatory cells and other mediators from mast cells, leading to inflammation and tissue damage
Describe the mechanism of a type III hypersensitivity reaction. Anitigen-antibody complexes activate complement, attracting neutrophils that release lysosomal enzymes
A patient has thymic aplasia in the setting of DiGeorge syndrome. For what 5 types of viral infections is the patient at risk? CMV, EBV, JCV, VZV, and chronic GI/respiratory viruses
What autoantibodies are associated with limited scleroderma/CREST syndrome? Anticentromere antibodies
Name the gene affected in Bruton agammaglobulinemia. BTK, which encodes for Bruton tyrosine kinase
Created by: ema1437



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