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Path - Chapter 6
Path
| Question | Answer |
|---|---|
| Binding of TLR to either PAMPS or DAMPS (danger associated molecular patterns) will send a signal to activate transcription factors, notably this one? | NF-kB (leads to cytokine production) |
| The enzyme that mediates the somatic recombination of T cell receptors is a product of which two genes? | RAG 1 and RAG 2 |
| What key facts allow us to detect lymphoid tumors based on analysis of antigen receptor gene rearrangement in cells? | All cells of the body have TCR genes but are unrearranged (presence of rearranged indicates T-cell lineage); Each T-cell is unique and thus one can test the DNA to see if a collection is polyclonal or monoclonal (neoplastic) |
| The TCR complex is a combination of the TCR alpha and Beta chains and these two sets of complexes (a total of 5 polypeptide chains)? | CD3 and Zeta |
| T-cells that aggregate at epithelial surfaces and can recognize peptides, lipids and small molecules without an MHC complex have this kind of receptor (NOT Alpha/Beta)? | Gamma/Delta |
| What two invariant chains are associated with the BCR complex? | Iga and Igb |
| What receptor does EBV recognize and is the reason that EBV readily infects B cells? | type 2 complement receptor (CD21 or CR2) |
| What are the most important APC for initiationg primary T-cell response against protein antigens? | Dendritic cells |
| These cells play an important role in humoral immunity by first recognizing antigen bound to antiboy and complemetn and secondly presenting them to B cells and selecting those with the highest affinity? | Follicular Dendritic Cells |
| These two surface markers are commonly used to ID NK cells? | CD16 and CD54 (CD16 is IgG receptor) |
| What does NK-cell secreted IFN-gamma do? | Activates macrophage killing of ingested material |
| what cytokines activate NK cell proliferation, which one activates NK-cell killing? | IL-2 and IL-15 proliferate; IL-12 KILL |
| Which chromosome house the MHC complex in humans? | Chromosome 6 |
| The MHC class 2 binding cleft is formed by which two protein chains? | Alpha 1 and Beta 1domains |
| People who inherit HLA-B27 have a 90 fold increased risk of developing what disease? | Ankylosing Spondylitis |
| What are the three categories of HLA-associated dieases? | Inflammatory diseases; Autoimmune diseases; Inherited errors of metabolism |
| This disease, which causes virilization of female external organs is associated to the HLA-BW47 allele because it is mapped within the gene? | 21-hydroxylase deficiency (congenital adrenal hyperplasia) |
| What are the principle co-stimulators of T-cells, what recognizes them, and how are they stimulated to be expressed? | B7(signal 2) is recognized by CD28 on T-cells, and is upregulated after the innate immune system recognizes microbes. |
| What is the second signal for B-cells and Macrophages needed for their activation? | CD40-CD40L |
| What does IL-4 do, and which subset of CD4 helper cells secrete it? | Secreted by TH2, stimulates TH2 and B cell differentiation to secrete IgE |
| Which T helper subtype is a powerful recruiter of neutrophil and monocytes and play a major role in several inflammatory diseases? | TH-17 |
| This hypersensitivity type is mediated by TH2 cells, IgE antibodies and MAST cells producing mediators that act on vascular smooth muscle and induce inflammation? | TYPE 1 Hypersensitivity |
| This hypersensitivity resulst from the direct action of IgM and IgG antibodies binding to tissues and cells in the body resulting in their lysis or phagocytosis? | TYPE 2 hypersensitivity |
| This hypersensitivty is caused by IgG and IgM antibodies reacting to normally present antigens in the circulation and forming complexes which deposit on tissues and recruit leukocytes which damage the tissues? | TYPE 3 hypersensitivity |
| This hypersensitivity is a result of direct damage done by Th1, Th17 and CTLS? | TYPE 4 Hypersensitivity |
| Goodpasture syndrome and autoimmune hemolytic anemia are what types of hypersensitivity? | TYPE 2 |
| The arthrus reaction, serum sickness, and Systemic Lupus Erythmatosus are what kinds of hypersensitivity? | TYPE 3 |
| Rheumatoid arthritis and contact dermatitis are what kinds of hypersensitivity reactions? | TYPE 4 |
| What are the two phases of type 1 hypersensitivity? | immediate (minutes; vasodilation, vascular leakage and SM spasm or glandular secretion) or Late-phase (2-24 hrs; infiltration with eosinophils, neutrophils, basophils, monocytes, and CD4 T cells) |
| What are some secretagogues of MAST cells? | C5a and C3a; IL-8, coedien and morphine; adenosine, mellitin (bee venom), physical stimuli |
| TH1 or TH2 cells play a role in type 1 hypersensitivity reactions? | TH2 cells |
| What does IL-13 do (secreted by TH2 cells)? | enhance IgE production and acts on epithelial cells to stimulate mucus secretion |
| What are some of the preformed mediators released upon activation of the MAST cell? | Histamine, Heparin and enzymes (neutral proteases and acid proteases) |
| This leukotriene released from MAST cell upon activation of PLA2 and AA is very chemotactic for neutrophils, eosinophils and monocytes? | Leukotriene B4 |
| These leukotrienes are the most potent vasoactive and spasmogenic agents known, released by MAST cells upon activation? | Leukotriene C4 and D4 |
| These are some of the products yielded from release of Arachodonic Acid from MAST cells? | Prostaglandin D2, Leukotrienes B4, C4, D4 (NOT PAF) |
| Among the cells recruited during the late phase of type 1 hypersensitivity, which one is particularly important? | eosinophils (release major basic protein; cationic protein; contribute to amplification and maintenance of the inflammatory response) |
| Is susceptibility to immediate hypersensitivity reactions genetically determined? | Yes, termed Atopy |
| What is one hypothesis which attempts to explain why developed nations tend to have more type 1 hypersensitivty diseases? | Hygiene hypothesis |
| Can extremely small doses of antigen, like those used in allergen skin testing produce systemic anaphylaxis? | YES |
| A patient with low hematocrit levels is found to have reactive antibodies to antigens on the surface of his red blood cells (Rh antigen and I antigen) what is the likely diagnosis? | Autoimmune hemolytic anemia (type 2) |
| What is the antigen recognized by auto-antibodies in Myasthenia Gravis? What are some symptoms? | Acetylcholine receptor; Muscle weakness and paralysis |
| A patient complains of progressive muscle weakness and double-vision. Upon examination you see her eyelids are very low (ptosis). CT scan reveals a mass in the mediastinum. Her symptoms are relieved by administration of a short-acting anticholinesterase. | Myasthenia gravis (antibody to acetylcholine receptor; thymoma is common finding in CT scan) |
| This type 2 hypersensitivity is due to antigen recognized in the basement membranes of kidney glomeruli and lung; patients often develop dyspnea, cough, hemoptysis and acute glomerulonephritis? | Goodpaasture's syndrome |
| What is the antgen involved with in autoimmune thrombocytopenic purpura? | Gp2b:3a integrin on platelets (the same affected protein in Glanzmann Thrombasthenia) |
| Which size (small, medium or large) antibody/antigen complex are most pathogenic, and is the antibody in excess of the antigen or vice versa? | Medium sized complexes where the antigen is in slight excess |
| What is the main source of tissue injury caused by type 3 hypersensitivity reactions? | Inflammation (vasculitis, glomerulonephritis, arthritis) |
| What is the principle morphological manifestation of immune complex injury? | Acute necrotizing vasculitis producing Fibroid necrosis (smudgy eosinophilic deposit) |
| SLE, post-streptococcal glomerulonephritis, polyarteritis nodosa, and reactive arthritis are what type of hypersensitivity? | Type 3 |
| A person with a recent bout with streptococcal infection is now experiencing symptoms of nephritis, what type of hypersensitivity reaction is this? | Type 3 (antigen is planted in the glomerular basement membrane) |
| The inflammatory reaction of associated with Th1 cells is dominated by activated (neutrophils or macrophages) while Th17 response is dominated by (neutrophils or macrophages)? | Th1 |
| Which two cytokines secreted by APC promote T cell differentiation into Th1 subtype? | IL-12 and IFN-g |
| Which cytokines work together to promote T cell differentiation into Th17 cells? | IL-1, IL-6, IL-23 and TGF-B |
| What changes are seen from activated macrophages after TH1 cells release the cytokine IFN-g (type 4 hypersensitivity)? | Increase effective killing; increase MHC-2; secrete IL-1, TNF, chemokines and more IL-12 |
| An aggregate of epitheloid cells, usually surrounded by a collar of lymphocytes,is referred to as what? | Granuloma |
| This type 4 hypersensitivity disorder is in response to protein antigens in the CNS myelin? | Multiple Sclerosis |
| What are some examples of type 4 hypersensitivity diseases? | Diabetes type 1; MS; RA; Crohns; Peripheral neuropathy (guillen-barre); Contact Sensitivity |
| What is perivascular "cuffing"? | accumulation of CD4 Tcells and macrophages around venules in a type 4 hypersensitivity reaction |
| Which cells in the type 4 hypersensitivity reaction produce IL-17, and IL-22? | Th17 |
| What are the principle mehtods/ways that CTL cells kill target cells? | perforin (hole); Granzyme (cleave caspases); FASL (apoptosis) |
| This protein stimulates expression of some "peripheral tissue restricted" self antigens in the thymus and is thus critical for deletion of immature T cells; mutations in AIRE are responsible for autoimmune polyendocrinopathy? | AIRE |
| T-cells encountering self antgens in the periphery receive two signals from receptors homologous to CD28 resulting inanergy, what are these two receptors? | CTLA-4 and PD-1 |
| These cells are produced in the thymus and display CD25, and require Foxp3 and IL-2 for development and maintenance? | T-regulatory |
| Mutations of what transcription factor found in T-regulatory cells results in the human disease (IPEX)? | Foxp3 |
| What are some immune-privileged sites in the body? | testis, eye, and brain |
| These genes are implicated in RA and type 1 diabetes (one gene for these 2), and Crohns disease respectively | PTPN-22 and NOD-2 |
| What is epitope spreading? | A consequence of automiimune disease in which normally concealed epitopes are revealed by damage, furthering the persitence and progression of the disease |
| This disease (type 3) is a chronic often febrile illness caused by anti-nuclear antibodies which principally injure the skin, joints, kidney and serosal membrane? | SLE |
| SLE predominantly affects which sex more? | Women |
| What are the 4 categories of ANAs? | A-DNA, A- histone, A-non histone proteins bound to RNA, A- nucleolar antigens |
| What is the most widely used method for detecting ANA? | indirect immunofluorescence |
| What are the four patterns of Indirect Immunoflouresence seen with ANAs? | Homogenous or diffuse (chromatin, histone, DDNA); Rim or peripheral (DDNA); Speckled (non-DNA nuclear constituents); Nucleolar (RNA) |
| Antibodies against which two antigens are diagnostic of SLE? | DDNA, and Smith antigen |
| Lupus patients sometimes have a false positive result for what infectious disease? | Syphilis (due to cross reactivity between antiphospholipid antibody and cardiolipin antigen) |
| Patients with anti-phospholipid antibodies are prone to thrombosis or bleeding? | Thrombosis (despite it being called lupus anticoagulant) |
| What are some examples of environmental factors affecting SLE? | UV light (alter DNA, increase IL-1); Sex hormones; Drugs |
| Phagocytic cells that have engulfed the denatured nucleus of an injured cell and sometimes found in SLE patients periardial and pleural effusions are called what? | LE cell |
| Describe the basic model for pathogenesis of SLE? | initial injury releases DNA from cells (UV light); Poor clearance of DNA (inherant problem with Complement); poor self-tolerance of B/T cells (self reactant); stimulation of lymphocytes and production of autoantibodies; Complex formation; tissue injury |
| The most characteristic lesions of SLE patients are due to complex deposition in what areas of the body? | blood vessels, kidney, connective tissue, and skin |
| What occurs to the vessel walls in chronic stages of SLE? | fibrinoid deposits (acute necrotizing vasculitis) |
| This is seen in 10 - 25 % of SLE patients and is characterized by mesangial cell proliferation and immune complex deposition without involvement of glomerular capillaries? | Mesangial lupus glomerulonephritis (class 1 and class 2) |
| This class of lupus nephritis is defined by less than 50% involvement of all glomeruli; complex deposits are primarily subendothelial and may create a "wire-loop" lesion? | Class 3 focal proliferative glomerulonephritis |
| This is the most severe form of lupus nephritis and pathologically identical to focal proliferative but occurs with > 50% of all glomeruli; Patient is symptomatic and produces hematuria and protenuria? | Diffuse proliferative glomerulonephritis (class 4) |
| This class of lupus nephritis is characterized by diffuse thickening of the capillary walls and is usually accompanied by nephrotic syndrome and sever proteinuria; deposits are primarily subepithelial? | Membranous glomerulonephritis |
| These lesions are formed due to active deposition of immune complex in subendothelial locations in type 3 and type 4 lupus nephritis? | "wire-loop" lesions |
| T/F In SLE patients changes in the interstitium and tubules are frequently present? | TRUE; |
| Facial malar rash (butterfly area of face) is seen in 50% of SLE patients and is typically incited by exposure to what? | Sunlight |
| What kind of joint involvement is seen in SLE patients? | NON-erosive synoviits with little deformity |
| What kind of valvular abnormality is sometimes seen in LUPUS? | Libman Sack endocarditis (NONbacterial verrucous endocarditis) |
| How is Libman Sacks (nonbacterial verrucous endocardidtis) different from infective endocarditis and rheumatic heart disease? | Smaller than infective; Larger than rheumatic heart disease as well as RHD is localized to the lines of closure |
| What are some features in SLE patient's spleen? | splenomegaly, capsular thickening and follicular hyperplasia (onion-skin lesions) |
| These, found in the bone marrow are strongly indicative of SLE? | LE or hematoxylin bodies |
| What is the most common cause of death in SLE? | renal failure and incurrent infections |
| This disease is characterized by skin plaques showing various degrees of edema, erythema, scaliness, follicular plugging and skin atrophy surrounded by an elevated erythmatous border; antibodies to double stranded DNA are rarely present? | Chronic discoid lupus erythmatosus |
| This disease is presents with non-scarring widespread rash along with mild systemc symptoms; antibodies to SS-A antigen are often present? | Subacute cutaneous lupus eryhtmatosus |
| This disease has ANA formation, multiple organ involvement, extremely high ANA specific for HISTONE; but rarely CNS and renal involvement and no Double DNA antibody? | Drug-induced lupus erythmatosus |
| This chronic disease is characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) resulting from immunologically mediated destruction of the lacrimal and salivary glands? | Sjogren Syndrome |
| These two antibodies (ANA) are considered serological markers of Sjorgen Syndrome? | SS-A and SS-B |
| What is the earliest histological finding in Sjorgen Syndrome? | Perivascular and periductal lymphocytic inflitration |
| What is needed for a diagnosis of Sjorgen syndrome? | biopsy of the lips |
| A patient complains of persistent nosebleeds, blurring of visions, and dryness of eyes. You examine and find her parotid gland is enlarged, WBC is very high (possible lymphoma), and her antibodies to SS-A/SS-B are very high. What is a likely diagnosis? | Sjorgen Syndrome |
| T/F Sjorgen syndrome patients are at high risk for developing B-Cell lymphoma due to a growth advantage imparted by somatic mutations? | TRUE; (marginal zone lymphoma) |
| This is a chronic disease chracterized by chronic inflammation, widespread damage to small blood vessels, and progressive interstitial and perivascular fibrosis in the skin and multiple organs? | Systemic sclerosis (scleroderma) |
| What are the two major clssifications of systemic sclerosis? | diffuse (widespread skin at onset with rapid progression and early involvement); limited (confined to fingers, forearms and face with visceral involvement occuring late) |
| What is the CREST syndrome? | occurs in patients with limited systemic sclerosis; Calcinosis, Raynauds phenomenon (discoloration of fingers/toes); esophageal dysmotility; sclerodactyly; telangiectasia |
| What are the two specific auto-antibodies found in systemic sclerosis? | Anti - topoisomerase 1 (diffuse); anticentromere antibody (CREST) |
| What Th2 cytokines are involved in fibroblast stimulation and resulting fibrosis in systemic sclerosis? | TGF-B and IL-13 |
| What occurs to the alimentary tract in patients with systemic sclerosis? | Collagenous fibrous replacement of the muscularis (esophagus); inflexibility of esophageal sphincter leads to GERD and Barrets; Loss of microvilli and villi lead to malabsorption |
| Is there any joint destruction in patients with systemic sclerosis? | not common as in RA |
| This is seen in virtually all patients with systemic sclerosis and is a result of episodic vasoconstriction of the arteris and arterioles of the extremeties, resulting in discoloration? | Raynauds |
| What population is more severely affected by systemic sclerosis? | black women |
| What is the most ominous manifestation of systemic sclerosis? | malignant hypertension (leads to renal failure and death) |
| A patient complains of difficulty swallowing, with some weight loss over the past few months. Her extremities have discoloration and skin thickening. She stated it's been hard to move her fingers. Testing reveals autocentromere antibodies. What is likely? | Systemic sclerosis |
| 4 out of these 11 criteria must be evident for the diagnosis of SLE? | Malar rash; discoid rash; photosensitivity; oral ulcers; arthritis; serositis; renal disorder; neurologial disorder; hematological disorder; immunological disorder; antinuclear antibody |
| This disease is characterized by high titers of antibodies to ribonucleoprotein particle-containing U1 ribonucloeprotein; shows good response to corticosteroids, and over time may evolve into classical SLE or systemic sclerosis? | mixed connective tissue disease |
| In the direct pathway of T-cell mediated graft rejection, what are the T-Cells recognizing? | Allogeneic (donor) dendritic cells (their MHC molecules MIMIC self+foreign) |
| Which pathway do the host's APC present graft antigen to T-cells? | Indirect pathway |
| Which pathway of cell-mediated graft rejection (indirect or direct) is where the CD8+ T-cells kill grafts cells? | Only in the direct pathway (cells are the donor cells which are getting killed) |
| Which pathway of cell-mediated graft rejection is the major pathway of acute rejection, which on does chronic rejection? | Direct |
| What is the cause of hyperacute graft rejection? | Preformed antibodies (past blood transfusions; multiparous women) |
| Kidney rejection reactions are classified as these three forms? | hyperacute, acute, and chronic |
| Histologically, what is the difference between humoral and cellular rejection? | Humoral rejection is associated with vasculitis; Cellular rejection is marked by interstitial mononuclear cell infiltrate |
| Endothelitis is seen with what type of rejection, and what causes it? | Acute cellular rejection due to infiltrating CD8+ T cells injuring vascular endothelium |
| Deposition of Cd4 in allografts is a strong indicator for what kind of graft rejection? | Acute humoral (rejection vasculitis) |
| A patient has had a kidney transplant and a biopsy reveals necrotizing vasculitis, narrowing of the arterioles. Studies reveal high levels of deposited Cd4, what should the patient be treated with (cyclosporin or B cell -depleting agents)? | This is acute humoral - B cell depleting agents |
| A patient with a kidney transplant 4 months ago has had progressivly rising creatinine levels. A biopsy reveals interstitial fibrosis, tubular atrophy, shrinkage of renal parenchyma, as well as graft arteriosclerosis. What kind of rejection is occuring? | Chronic rejection |
| Neutrophilic accumulation within arterioles, thrombotic occlusion of glomerular cappilaries, fibrinoid necrosis, kidney cortex infarction (what kind of rejection)? | Hyperacute |
| Extensive interstitial mononuclear cell infiltration, mild interstitial hemorrhage, endothelitis (swollen endothelial cells) (what kind of rejection)? | Acute cellular |
| necrotizing vasculitis, neutrophilic infiltration, thickening of the intima with proliferating fibroblasts, myocytes, and foamy macrophages. (what kind of rejection)? | Acute humoral |
| How does cyclosporine work? | blocks activation of NFAT which leads to transcribe IL-2 |
| What are the pros and cons of depleting donor T cells before transfusion of bone marrow to patients? | Pro: elimination of GVH disease CON: increased incidence of graft failure, EBV related B-cell lymphoma, recurrence of disease in leukemic patients increase |
| genetic deficiencies in T cells are often clinically indistinguishable from deficiencies in both T cells and B cells T/F? | TRUE; T cells mediate B cell antibody synthesis |
| This x-linked disease is characterized by failure of pro-B cells and Pre-B cells to develop into mature B cells? | X-linked Agammaglobulinemia |
| What is the molecular basis of X-linked agammaglobulinemia? | mutations in the btk tyriosine kinase prevents signals from the BCR (pre-B cell; with the heavy chain complexed with the surrogat elight chain) from sending signal down to initiate light chain rearrangement and maturation stops at pre-BCR |
| At what age does Agammaglobulinemia manifest? | 6 months due to depletion of maternal Igs |
| What organisms are typically insiduous in patients with agammaglobulinemia? | Haempphilus influenza, streptococcus pneumonia, and staphyloccocus aureus, enteroviruses (polio) and Giardia Lamblia |
| A young buy of 6 months comes in with recurrent otitis media and pneumonia, cultures grow staphylcoccus aureas. The child's Ig count is low for all Igs, what is a likely diagnosis, FACS analysis of bone marrow reveals large numbers of CD19 tagged cells? | X-linked agammaglobulinemia (CD19 is a marker for PRE-B cells) |
| A 3-year old girl comes in with recurrent ear infection and a history of pnemonia & bronchitis. B/T cell counts are normal. Circulating Igs are down. Biopsy of her LNs reveals hyperplastic germinal centers. She has no circulating plasma cells. | Common variable immunodeficiency (affects both sexes, similar to x-linked agammaglobulinemia, uknown cause) |
| This disease is marked by extremely low levels of serum and secretory IgA, recurrent infections of mucosal systems (respiratory, gastrointestinal, and urogenital) and increased frequency of autoimmune disorders such as SLE and RA? | Isolated IgA deficiency |
| This disease is characterized by reduced IgA, IgG, and IgE antibodies and is caused by defieiciency in either CD40L (x-linked) or AID (auto-recessive)? | Hyper-IgM |
| Why are patients with CD40L mutations susceptible to the intracellular organism pneumocystis jiroveci (causes pneumonia)? | CD40L(hyper-IgM) is also involved with cell mediated immunity |
| This disease is a result from the failure of development of the third and fourth pharyngeal pouches resulting in thymic aplasia, tetany, and congenital heart defects? | DiGeorges Syndrome |
| This patient comes in with recurrent pneumonia caused by pneumocystis jeroveci. Cell counts reveal normal B cell numbers but low T cells. Lab values also show decreased calcium levels. And a murmur is noted on heart exam. What is a likely diagnosis? | DiGeorges Syndrome |
| Patient present with thrush, diaper rash and failure to thrive. Morbilliform rash occur after birth due to GVH disease by maternal T cells, recurrent infections from pathogens such as candida albicans, pnemocystis jiroveci, cytomegalovirus, psuedomonas. | SCID [Can be caused by ADA deficiency (auto-recessive) or common gamma chain subunit deficiency (x-linked)] |
| This disease is caused by mutations in transcription factors that are required for MHC class 2 gene expression; results in SCID? | bare lymphocyte syndrome |
| What do mutations in the common gamma chain subunit of cytokine receptors lead to ? | X-linked SCID |
| What do mutations of the enzyme adenosine deaminase lead to ? | Autosomal recessive SCID |
| This X-linked immunodeficiency is marked by thrombocytopenia, eczema, and marked vulnerability to recurrent infection, ending in early death. Gene defect in WASP, a protein that links antigen receptors to cytoskeleton? | Wiskott-Aldrich Syndrome |
| What is the problem associated with a deficiency in the classical complement pathway? | increased incidence of SLE-like autoimmune disease |
| What organism thrives in patients that cannot form the membrane attack complex of the complement system? | Neisseria bacteria (thin walled) |
| What does deficiency with the C1 inhibitor give rise to and why? | hereditary angioedema (increase in C1r and C1 s, Factor 12, results in increased vasoactive components >> edema) |
| A defect in enzymes required for glycophosphatidyl inositol linkages (which are essential for assembly of decay-accelerating factor and CD59, both of which regulate complement) lead to uncontrolled complement activation and hemolysis? | Paroxysmal Nocturnal Hemoglobinuria |
| What are the five groups at risk for developing AIDS in order of most common? | Homosexuals; IV drug abusers; Hemophiliacs; Blood transfusee; Heterosexuals |
| What are the three major routes of transmission of HIV? | Sexual, paraenteral (IV drugs, hemophiliacs); mother to infant |
| Viral transmission of HIV during sexual contact occurs in two ways? | direct innoculation into a blood vessel breached by trauma; infection of dendritic cells or CD4 cells in mucosa |
| What concurrent conditions result in increased risk of HIV transmission during sexual contact? | Any STD (especially those that result in genital ulceration) |
| What are the three routes of transmission of HIV from mother to infant? | in utero by transplacental spread; during delivery; breast milk |
| What is the target antigen on HIV targeted by antibodies used in detection studies like ELISA? | p24 |
| What are the two major (systems) targets of HIV? | Immune system and Central Nervous System |
| What does the HIV molecule use as a receptor and co-receptor to infect cells? | CD4 and CCR5 and CXCR4 (chemokines are co-receptors) |
| The initial step of HIV infection is binding of this HIV surface glycoprotein with CD4 molecules? | gp120 |
| What happens after gp120 binds CD4? | gp120 then changes to allow binding of CCR5, which leads to a change on gp41 allowing the fusion of virus with host cell |
| After incorporation of the HIV DNA into Tcell genome, how exactly is the proviral DNA transcribed? | Activation of T cell leads to production o ftranscription factor NF-kB which also binds to the HIV proviral DNA segemnt on the genome, allowing transcription |
| What are some mechanisms of T cell death caused by HIV? | Direct killing; destruction of lymphoid organs; activation induced cell death; loss of immature precursors; giant cell formation (t-tropic virus); apoptosis of uninfected T cells by binding of soluble gp120 |
| What are some Non T cells capable of being infected with HIV? | macrophages (mostly tissue);mucosal dendritic cells (present to lymphoid organs); follicular dendritic cells (reservoir with HIV on surface) |
| What are some causes of polyclonal activation of B cells following HIV infection? | cytolomegavirus or EBV reactivation; gp41 is a prominent B cell growth factor; IL-6 from HIV infected macrophages |
| What is responsible for the initial containment of HIV infection? | Anti HIV CTL |
| What is the primary clinical measurement used to assess when to atart anti-retroviral therapy and what is a good clinical marker for HIV disease progression following Acute infection? | CD4 cells; Extent of viremia ("set point") |
| This is the most common fungal infection in patients with aids, and can sometimes be a marker for immune decompensation? | Candiadis |
| T/F Tuberculosis like all atypical mycobacteria typically occur late in AIDS under severe immunocomprimise? | FLASE; tuberculosis can occur early in AIDS |
| What is the major clinical manifestation of cryptococcosis seen in AIDS? | meningitis |
| This protozoa causes encephalitis and is responsible for 50% of all mass lesions in the CNS (prominent in Immunocomprimised)? | toxoplasma gondii |
| Persistent diarrhea seen in untreated AIDS patients is caused by these infectious agents? | Cryptosporidium; isopora belli, microsporidium and enteric bacteria (salmonella, shigella) |
| What is common about the malignancies developed by AIDS patients (kaposi sarcoma, B cell ymphoma, cervical and anal carcinoma)? | Caused primarily by oncogenic DNA viruses |
| A vascular tumor and the most common neoplasm in patients with AIDS, it is caused KSHV or human herpes virus 8; involves prolif. of mesenchymal spindle cells and prolif. of slit-like vascular spaces; affects skin, mucous m., GI, lung, and lymph nodes | Kaposi sarcoma |
| What are the three groups of lymphomas in AIDS patiens? | Systemic, primary CNS, Body cavit |
| What are some clinical innovations recently implemented that has resulted in fewer mortality, and less opportunistic infections as well as previously associated diseases such as Kaposi's Sarcoma? | HAART (highly active antiretroviral therapy) |
| This is the paradoxical deterioration of the immune system during administration of HAART in patients with AIDS. | immune reconstitution inflammatory syndrome |
| What are some complications of AIDS HAART therapy? | Immune reconstitution inflammatory syndrome; Long term toxicity; Non-AIDS morbidity (i.e cancer) |
| Biopsies of enlarged lymph nodes in the early stages of HIV infection reveal what? | Marked follicular hyperplasia; attenuated mantle zones; (merging germinal centers with interfollicular areas) |
| With electron microscopy and in situ hybridization, where can HIV particles be found in the lymph nodes? | In the nodes, they are concentrated on follicular dendritic cells ( trapped by immune complex) |
| In early HIV patients, what do peripheral blood smears show (caused by the B cell hyperplasia and resultant hypergammaglobulinemia)? | Rouleax formation |
| What morphological changes occur within lymph nodes as HIV progresses and the B-cell proliferation subsides? | Depletion of follicles; disruption of the organized network of follicular dendritic cells; germinal centers may be hyalinized |
| What stain is used to detect amyloid deposits? | Congo red (pink/red color OR green briefringence) |
| What is the structure of Amyloid? | continuous non-branching fibrils with cross-B-sheet conformation |
| What are the three most common forms of amyloid proteins? | AL (amyloid light from Ig light chains); AA (from liver); AB (Alzheimers) |
| Which amyloid deposition is associated with plasma cell tumors? | AL amyloid (made of free light chain) |
| This form of amyloid fibril protein is derived from SAA during acute phase responses? | AA amyloid (often called secondary amyloidosis) |
| This amyloid protein is made from proteolysis of amyloid precursor protein and is often found at the core of cerbral plaques in Alzheimer patients? | B-amyloid protein (AB) |
| This mutated protein is seen in familial amyloid polynueropathies and deposited in heartrs of senile systemic amyloidosis? | transthyretin |
| What are the two categories of proteins that form amyloid? | normal proteins with tendency to misfold are overproduced; mutations of normal proteins (TTR transthyretin) |
| This form of amyloidosis is due to some form of underlying plasma cell dyscrasia; is seen in som multiple myeloma patients; and sometimes associated with Bence-Jones proteins (Light chains)? | Immunocyte dyscrasia with amyloidosis (primary) |
| This form of amylodosis is associated with chronic inflammatory conditions (RA, ankylosing spondylitis, IBS, ulcerative colitis, and crohns) as well as chronic skin infections associated with "skin-popping" of narcotics? | Reactive systemic amylodosis (AA protein) |
| A patient with kidney failure presents with carpal tunnel syndrome, she has been on hemodialysis for a months, what is the underlying problme? | Hemodyalisis-associated amyloidosis (B2 microglobulin cannot be filtered and deposits) |
| This hereditary amyloidosis is due to AA fibril deposits, and is caused by an autoinflammatory syndrome due to abnormally high levels of IL-1; and characterized clinically by recurrent fever and serosal inflammation? | Familial mediterranean fever (pyrin gene) |
| Amyloid deposition of the kidney results in what? | Capillary lumen obliteration (which may lead to ischemia) |
| Amyloid deposition in the spleen takes two forms, what are they? | Sago spleen (tapioca like granules in Follicles); Lardaceous spleen (walls of splenic sinus in red pulp) |
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