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Flashcards

immunodeficiency

lumen7 Immunopathology Inglês 42 flashcards

Flashcards do conjunto (42)

  1. 1cyclophosphamideacts by covalent alkylation with active metabolites, cross links with DNA, result marrow toxicity-->lymphocyte numbers /function
  2. 2inherited complement defsrare, most common are C3 and C2, if C5 through C9 no MAC formed
  3. 3B cell (humoral) defs1. brutons x linked agammaglobulinaemia 2. common variable immunodef CVID 3. hyper IgM immunodef
  4. 4brutons x linked agamaglobulinaemia symp dx and txsymp: normal first 6 months then recurrent pyogenic infections, susp to giardia, dx: low b cell count tx: antibiotics and monthy gammaglobulin transfusions
  5. 5Hyper IgM immunodeficiency1. rare x linked 2. defect gene coding for CD40 ligand essential to class switching 3.
  6. 6Hyper IgM symp dx + txsymp: boys, bacterial infections. dx: def IgG and IgA, no CD40 ligand. tx: antibiotic and gammaglobulin transfusions
  7. 73 combined B and T cell defs1. SCID 2. ADA adenisine deanimase def 3. xlinked SCID
  8. 8ADA (adenosine deanimase) def symp dx &txsymp: all types infections. dx: low peripheral T count, detection of ADA, low serum levels of 3 major Igs. tx: bone marrow transplant, gene tx,
  9. 9x linked SCIDlethal. defect in gene for gamma chain of IL-2 receptor. Need bone marrow transplant or gene tx
  10. 10types of acquired deficiencies1. malnutrition. 2. cancer. 3. stress. 4. exercise. 5. v young/old. 6. immunosupression 7. infections
  11. 11malnutritionimpaired functions due to lack of selenium copper and fe, lymphoid system particular affected, worldwide issue
  12. 12symptoms dx and tx of complement defssymp: recurrent bacterial infections, dx: symp + CH50 levels, tx: antibiotics
  13. 13glucocortcoidsinhibition of b cells, t cells and cytokines
  14. 14AIDS statsleading cause of death in africa. ~1 million dx in US.
  15. 15HIV symp dx1.70-80% asymptomatic 3-12 weeks after exposure, 2. fever, rash, cervical lymphadenopathy, aseptic meningitis, encephalitis, myelitis, polyneuritis, 3. fall in CD4 counts to 300-400, 4. depletion of CD4 cells leaves susp to infections, 5. lymphatic tissue is main reservoir of HIV
  16. 16list of x linked immunodef diseases (5)1. CGD, 2. wiskott aldrich syndrome, 3. SCID, 4. x linked agammaglobulinaemia, 5. hyper IgM immunodef
  17. 17defect in CGDintracellular phagocytic killing and inability to produce bacterial cytolysins
  18. 18defect in wiskott aldrich syndromethrombocytopenia and low serum IgM, low CD23 expression causes abnormal B cell development
  19. 19defect in SCIDdefect in both humoral and cel mediated immune responses
  20. 20defect in Hyper IgMdefective CD40 ligand on lymphocytes prevent isotope switching therefore low serum levels of all immunoglobulin classes except IgM
  21. 21four components of immune systemT cells, B cells, phagocytes, complement
  22. 22Diagnosis of LADdetection of defect in CD11 and CD18
  23. 232 types immunodeficiencycongenital or acquired
  24. 24primary (congenital)defects in structural components or developmental stages of immune system. May be masked at birth by abs from mother.
  25. 25causes of secondary (acquired) immunodeficiency1. infectious disease (HIV) 2. cancer 3. stress 4. excessive exercise 5. malnutrition 6. drug tx
  26. 26warning signs of primary immunodef1. family hx 2. recurrent serious infections 3. infant failure to thrive 4. persistent thrush 5. need for IV antibiotics
  27. 27who gets repeated bacterial infections?pts with:1. low phagocyte numbers (or defective) 2. complement defects 3. humoral immunity defects
  28. 28defects in CMI (cell mediated immunity) usually result in recurrent......fungal protozoan intracellular bacterial and viral infections
  29. 29phagocytic dysfunction1 low neutrophil numbers from defective production during haemopoieses, 2. life threatening, 3. tx=recombinant granulocyte colony stimulating factrs
  30. 30The WHO classifies immunodeficiencies into 5 groups1. phagocytic dysfunction, 2. complement ab+def 3. B cell (humoral) ab+def 4. T cell (CMI) ab+def 5. combined B&T cell ab+def
  31. 31leukocyte adhesion defs (LADs)LFA-1 is two chain molecule that allows neutrophils to stick to endothelial wall. If either chain defective, cannot migrate to site of infection
  32. 32symptoms of LAD1. recurrent bacterial infections 2. absence of neutrophils at infected sites leading to large abcesses
  33. 33why is the cause of immundef sometimes hard to identify?1. normal values have wide range, 2. complex inter relationships between components, 3. sometimes difficult/expensive to test for
  34. 34treatment for LADantibiotics
  35. 35chronic granulomatous disease CGDNADPH oxidase is defective=no superoxide ions to kill intracellular bacteria
  36. 36what is most common cause for CGDdefect in cytochrome b
  37. 37what is future tx for cgdgene replacement therapy
  38. 38Dx of CGDneutrophils stained with nitro-blue tetrazolium remain pale yellow rather than blue
  39. 39Tx of CGDantibiotics
  40. 40Chediak-Higashi syndrome1.autosomal recessive mutation in LYST protein 2. recurrent bacterial infections 3. partial albinism 4. phagocyte granules impotent 5. infiltration of organs by lymphocytes
  41. 41three diseases associated with defective intracellular killing of ingested bacteria1. chronic granulomatous disease CGD 2. Chediak Higashi syndrome 3. macrophage defects in IFN signalling
  42. 42macrophage defects in IFNy signallingautosomal recesive, fatal unless tx with IFNy

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Immunopathology FlashCards sobre immunodeficiency, criado por lumen7 em 05-05-2013.
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lumen7
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