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Immunology
FA review Round 1 2020
| Question | Answer |
|---|---|
| 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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