Immunology Flashcards
Swelling and inflammation 24 to 48 hours after a protein inoculation is what kind of reaction
Type III HS aka Arthus reaction
Preexisting antibodies react with the injected antigen, forming immune complexes, which in turn activate complement and cause inflammation.
thrombocytopenia, immune deficiency, and eczema
nosebleeds
(hint X recessive)
Wiskott aldrich syndrome
Typically, IgM is low, and IgE and IgA are increased.
Mutations in WASP gene, which is a cytoskeletal glycoprotein
Multiple pyogenic infections requiring antibiotics and a negative nitroblue tetrazolium test is indicative of what disease?
Chronic granulomatous disease: an inherited deficiency in the production of NADPH oxidase.
A positive nitroblue tetrazolium test (NBT) determines the reduction of NBT into a blue substance (Formazan). In the absence of NADPH oxidase, the dye does not turn a blue and the test is read as negative and confirms the diagnosis of chronic granulomatous disease (CGD)
more specific and robust test includes the dihydrorhodamine (DHR) flow cytometry test. This test evaluates NADPH oxidative function in neutrophils. Mixing sample neutrophils with DHR and then activating them with phorbol myristate acetate (PMA) results in oxidation of DHR to a fluorescent compound, rhodamine 123, which can be measured by flow cytometry
thrombocytopenia, eczema, and immunodeficiency is indicative of what disease?
what levels of immunoglobulin will you see?
What is the inheritance pattern?
Wiskott-Aldrich syndrome
Elevated levels of IgA and IgE in the absence of IgM
X-linked inheritance
polymyositis autoantibodies? How do you differentiate it from other muscle disease (dermatomyositis)?
antinuclear (ANA), andti-histidyl-tRNA synthetase (anti-Jo-1) are present in most cases.
Biopsy shows endomysial mononuclear inflammatory infiltrate and patchy muscle fiber necrosis. and lack of skin involvement.
What is the function of calcineurin?
what drugs inhibit calcineurin activation?
essential for the activation of IL2 (which promotes differentiation of T cells)
APC binding with Tcell can activate calcineurin which will dephosphorylate NFAT allowing NFAT to enter nucleus and bind IL2 promoter–>transcription of IL2
Cyclosporine and tacrolimus are commonly used immunosuppressants used in transplant patients that inhibit calcineurin activation.
Cytogenic defect t(15;17) is associated with what disease and what are the specific translocations?
APML
translocation involving the retinoic acid receptor alpha (RARA) from ch 17 and the promyelocytic leukemia (PML) gene on 15 leads to PML/RARA, a fusion gene whose product inhibits differentiation of myeloblasts and triggers the development of APML
Pt’s with IgA deficiency are particularly susceptible to what infection causing diarrhea?
Giardia
pear shaped - multiple flagella - 2 nuclei
Neurologic abnormalities, partial albinism, and immunodeficiency-what dz? what inheritance?
Chedak-Higashi syndrome auto rec disorder of neutrophil phagolysosome fusion
young male presents with morning stiffness and low back pain. Xray shows narrowing of SI joints. Dz? genetic association?
Ankylosing spondylitis
seronegative spondyloarthropathies, pt’s with this dz have higher incidence of HLA B27 allele
Triad of hemolytic anemia, hypercoagubility, and pancytopenia with CD55 and CD59 deficiency suggests what disorder?
Paroxysmal nocturnal hemoglobinuria (PNH)
PIGA gene
CD55 (decay accelerating factor) and CD59 (MAC inhibitory protein) help keep complement in check and block MAC from forming on normal cells. Without them –> pancytopenia
