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Immunology

FA review Round 1 2020

QuestionAnswer
In Lambert-Eaton disease, what is attacked by antibodies? Presynaptic voltage-gated Calcium channels
Which autoimmune disease is due to antibodies against Pre-synaptic voltage-gated Calcium channels in the NMJ? Lambert-Eaton disease
If the muscular debilitating condition is of autoimmune origin and is seen to improve with repetitive nerve and muscle stimulation, what is the most likely diagnosis? Lambert-Eaton disease
If symptoms improve with repetitive nerve and muscle stimulation. Dx? Lambert-Eaton disease
If symptoms worsen with repetitive nerve and muscle stimulation. Dx? Myasthenia Gravis
What is affected by antibodies in Myasthenia gravis? Postsynaptic ACh receptors
If the autoimmune condition is directed to Postsynaptic ACh receptors of the NMJ, what is the most likely diagnosis? Myasthenia Gravis
Pre- or Post-synaptic Calcium channels in Lambert Eaton syndrome? Pre-synaptic
Pre- or Post-synaptic ACh receptors in Myasthenia Gravis? Post-synaptic
Which channels or receptors are affected in Lambert-Eaton, Calcium channels or ACh receptors? Calcium channels
ACh receptors or Calcium channels affected in Myasthenia gravis? ACh receptors
In the anatomy of an antibody, which part is the binding site for immunoglobulins, such as IgG or IgM? CH2 component of the Fc fragment
What can be thought to attach to the CH2 component of the Fc fragment of an antibody? IgG and IgM (immunoglobulins)
What are the two main divisions of an antibody? Fc portion and Fab portion
What does the CH3 component of the antibody serves as binding site for? Macrophages
Macrophage attachment to the CH3 component of Fc fragment of an antibody enhances ____________________. Phagocytosis
What are the two (abbreviated) components of the Fab fragment of an antibody? VL and VH components
The VL and VH serve as binding sites for the: Antigens
To which part of the antibody would IgM or IgG be binding to? CH2 component of the Fc fragment
VL and VH together dictate --> Antigen binding specificity
What is the pathogenesis of Anaphylactic shock? Cross-linking of IgE present on the immunoglobulin surface receptors, on both mast cells and basophils, lead to release of vasoactive compounds
IgE surface receptors cross-linkage lead to : Release of vasoactive compounds leading to anaphylactic shock
Which immunoglobulin's surface receptors are involved in anaphylactic shock? IgE
Which cells have IgE (immunoglobulin) surface receptors, that may cross link and produce an anaphylactic shock reaction? Mast cells and Basophils
What is a Hapten? Molecule that cannot elicit an immune response on its own but can do it when a protein is bound to it
If a compound or molecule cannot create an immune response by itself, but once bound to a protein, it produces an immune response, the compound is known as: Hapten
What is a common medication that is often used as a Hapten? Penicillin
What condition is penicillin-induced, due to penicillin been a hapten molecule? Autoimmune Hemolytic anemia
Inflammatory arthritis associated with dermatology scales Psoriatic arthritis
What is the the common presentation of Psoriatic arthritis? Asymmetric arthritis and inflammation of other without with psoriasis
What is strong nail-manifestation seen in Psoriatic arthritis? Nail pitting
Nail pitting is strongly associated with which Seronegative Spondyloarthritis? Psoriatic arthritis
Dactylitis and "pencil-in-cup" deformity of DIP joints is often seen with: Psoriatic arthritis
List of the most common Paraneoplastic syndromes associated with Thymoma: 1. Myasthenia gravis 2. Pure RBC aplasia 3. Hypogammaglobulinemia
What are the ocular deficits seen with Myasthenia gravis? Ptosis and diplopia
Which neoplasm is strongly associated with Myasthenia gravis? Thymoma
What is the feared or serious adverse effect of Cyclosporine? Nephrotoxicity
What is a common medication used to prevent GVHD in bone marrow transplants, that can affect the kidneys? Cyclosporine
What happens to the cell that is in a state of anergy? Cell is refractory to any further stimulation
A T-cell without the second signal for activation is said to be: In a refractory state to any further stimulation
If the T-cell in question is non-responsive to stimulation, it may be due to no __________________________. Signal 2 in T cell activation
What is the second signal in T cell activation? Co-stimulation via interaction o B7 on APC and CD28 on the T-cell
What part of the APC interacts with the T-cell in the second signal for its activation? B7 on APC interactions with CD28 on the T-cells
How does a state of anergy help the immune system? To maintain effective T cells and remove those self-tolerant or overactive T-cells
What are the main 3 clinical features of Job syndrome? 1. Recurrent Staphylococcal abscesses 2. Elevated IgE 3. Atopic dermatitis
What is the common mnemonic used to summarize the clinical features of Job syndrome? FATED F- Facies are coarse A- Abscesses that are cold T- retained primary Teeth E- increased IgE D- Dermatologic problems
Which immunodeficiency is seen with cold staphylococcal abscesses? Job syndrome
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Bruton Agammaglobulinemia B-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Selective IgA deficiency B-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? CVID B-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Thymic aplasia (Di George) T- cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? IL-12 receptor deficiency T-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Job syndrome T-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Chronic Mucocutaneous Candidiasis T-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? SCID T- & B-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Ataxia-Telangiectasia T- & B-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Hyper-IgM T- & B-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Wiskott-Aldrich syndrome T- & B-cell
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? LAD type 1 Phagocyte
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Chediak-Higashi syndrome Phagocyte
B-cell, T-cell, T-& B-cell, or Phagocyte Immunodeficiency? Chronic Granulomatous disease Phagocyte
Which immunodeficiency should be suspected in a young, male with Hx of recurrent infections, primarily bacterial by encapsulated organisms? Bruton agammaglobulinemia
To which type of bacterial organisms is a person with X-linked Bruton agammaglobulinemia most susceptible ? Encapsulated bacteria
What is the histological finding in a lymph node bx of Bruton agammaglobulinemia patient? No germinal centers
Why is it normal or expected to not find germinal centers in a B-cell immunodeficiency, during a lymph node biopsy inspection? Germinal centers are the location in the lymph node where B-cells mature through somatic hypermutation and immunoglobulin class switching
What are Peyer Patches? Gut lymphoid tissue, located primarily in the Ileum
Which part of the small intestine is rich in Peyer Patches? Ileum
What are the cells in Peyer Patches involved in presenting antigen to B cells? M cells
Where are M cells found? Peyer Patches
What is the common role for M cell in Peyer Patches? Take up antigens from intestinal tract and present to B cells
Once M cells present antigens to B cells in the Peyer patches, what immunoglobulin antibodies are secreted by B cells? IgA antibodies
What is the classic triad of Reactive arthritis? 1. Arthritis 2. Conjunctivitis 3. Urethritis
What is a common syndrome seen in Reactive Arthritis? Sacroiliitis
What bacteria is associated with Reactive arthritis? Shigella, Yersinia, Chlamydia, Campylobacter, and Salmonella
What are key differentiating factors that indicate Septic arthritis rather than Reactive arthritis? 1. Elevated WBC count (> 50, 000) in synovial fluid 2. Systemic symptoms
If patient present with arthritis, fever, and a elevated WBC count in synovial fluid, it most likely indicate Septic or Reactive arthritis? Septic arthritis
What is osteoarthritis? Condition of mechanical wear-and-tear on weight-bearing joints, which worsens with use
What is a key physical characteristic of Osteoarthritis? Morning stiffness lasting less than 30 minutes
Which are the hand joints involved in Osteoarthritis? DIP and PIP
What condition is associated with Heberden nodes? Osteoarthritis
Are Bouchard nodes associated with Osteoarthritis or Rheumatoid arthritis? Osteoarthritis
What are the Heberden nodes? Swelling of DIP joints
Swelling of DIP joints are know as: Heberden nodes
What is the name given to swelling of the PIP joints in Osteoarthritis (OA)? Bouchard nodes
What are 2 common examples of Passive immunity? 1. Transplacental delivery of IgG 2. Post-exposure prophylaxis with Hepatitis B antibodies
What is the definition of Passive immunity? Preformed antibodies that are received and confer temporary immunity to help fight off infections
What are examples of ACTIVE immunity? 1. Natural infection 2. Vaccines 3. Toxoid
Exposure to Exogenous antigens, is active or passive immunity? Active
What is Osteoarthritis? Condition of mechanical wear and tear on weight-bearing joints, which worsens with use and is characterized by morning stiffness of less than 30 mins
Does osteoarthritis get worse or better throughout the day? Worse
What are the classical finger findings of Osteoarthritis? Heberden nodes and Bouchard nodes
Which finger joints are affected in osteoarthritis? DIP and PIP
DIP and PIP are affected in osteoarthritis or rheumatoid arthritis? Osteoarthritis
What is the term given to swelling of the DIP joints in OA? Heberden nodes
What term is given swelling of the PIP joints in OA? Bouchard nodes
Two examples of Passive Immunity: 1. Transplacental delivery of IgG 2. Post-exposure prophylaxis with Hepatitis B antibodies
What is the definition of passive immunity? Preformed antibodies are received and convey TEMPORARY immunity to help fight infections
Is passive immunity permanent or temporary? Temporary immunity
What are examples of acquired active immunity? 1. Natural infection 2. Vaccines 3. Toxoid
Is passive or active immunity related to exposure to exogenous antigens? Active immunity
Natural infection. Passive or Active immunity? Active immunity
Vaccines. Passive or Active immunity? Active immunity
Toxoid exposure. Passive or Active immunity? Active immunity
What causes Hyper-IgM syndrome? Defect in antibody class switching, which leads to elevated IgM and low levels of other Immunoglobulins
What is the most common defect causing Hyper-IgM syndrome? Defective CD40-Ligand on TH cells
A defect in CD40-Ligand leads to which immunodeficiency? Hyper-IgM syndrome
What are clinical features of Hyper-IgM syndrome? 1. Severe pyogenic infections early in life 2. Opportunistic infections with Pneumocystis, Cryptosporidium, and CMV
Which condition is often associated with Birbeck granules? Langerhans Cell histiocytosis
What is a common histological finding of Langerhan cells in patients with Langerhans Cell histiocytosis? Birbeck granules
What are Langerhan cells? Type of APC found only in the skin
What are APCs? Cells that express MHC II, which binds to CD4+ T cells, providing the 1st signal for helper T-cell activation
What is provided by the APC expression of MHC II in regards to immunity? 1st signal in for TH cell activation
To which T-cell type do MHC II attach to? CD4+ T cells
Defective HFE gene. Dx? Hemochromatosis
What is the result of a defective HFE gene leading to Hemochromatosis? Increase efficiency in dietary iron absorption and hepatic storage
Which condition is characterized with an increased in dietary iron absorption and hepatic iron storage? Hemochromatosis
What lab levels are increased in Hemochromatosis? - Serum TRANSFERRIN saturation, - Serum Iron - Ferritin
Are serum iron, transferrin and ferritin increased or decreased in Hemochromatosis? Increased
What iron lab or homeostatic value is decreased in Hemochromatosis? Total Iron Binding Capacity (TIBC)
Is TIBC increased, decreased, or normal in Hemochromatosis? Decreased
Which cells mediate Acute Transplant rejection? Cytotoxic CD8+ T lymphocytes
What is the pathology of Acute Transplant rejection? Cytotoxic CD8+ T cells cause cytotoxic graft cell death and release of proinflammatory cytokines
What is the time frame for Acute Transplant rejection? Weeks to months
Which type of acute transplant rejection is similar to Hyperacute transplant rejection? Humoral mediated acute transplant rejection
What is the difference in Hyperacute and Humoral acute transplant rejection? The antibodies in hyperacute are performed, while in humoral acute rejection, the antibodies develop after the transplant
CD8+ and CD4+ T cells activated against DONOR MHC. What type of transplant rejection is described? Cellular mediated Acute Transplant Rejection
What are the characteristics of the pathogenesis of Acute Transplant rejection? Vasculitis of graf vessels with dense interstitial lymphocytic infiltrate
What is used to prevent or reverse effects of Acute Transplant rejection? Immunosuppressants
What is the pathogenesis of Chronic Transplant rejection? CD4+ T cells respond to recipient APCs preventing donor peptides , including allogeneic MHC
What is the main cytokine produced by TH1 cells? INF-gamma
What T cell type is known to produce INF-gamma? TH1- cell
What are some properties or effects of INF-gamma? Stimulate macrophage activity, antiviral and antitumor effects
Which junctions are attacked by autoantibodies in Pemphigus vulgaris? Desmosomes
Are Desmosomes or Hemidesmosomes attacked in Pemphigus vulgaris? Desmosomes
Autoimmune disease against the intercellular junctions between epidermal cells (desmosomes)? Pemphigus vulgaris
What are some features and/or symptoms of Pemphigus vulgaris? 1. (+) Nikolsky sign 2. IM --> Reticular (net-like) pattern around epidermal cells 3. Type II hypersensitivity
What is the IM pattern seen in Pemphigus vulgaris? Reticular or "net-like" pattern around epidermal cells
What type of hypersensitivity reaction is Pemphigus vulgaris? Type II
Is Pemphigus vulgaris seen with a (+) or (-) Nikolsky sign? Positive (+)
Created by: rakomi
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