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immunodeficiency
Fichas del conjunto (42)
- 1cyclophosphamideacts by covalent alkylation with active metabolites, cross links with DNA, result marrow toxicity-->lymphocyte numbers /function
- 2inherited complement defsrare, most common are C3 and C2, if C5 through C9 no MAC formed
- 3B cell (humoral) defs1. brutons x linked agammaglobulinaemia 2. common variable immunodef CVID 3. hyper IgM immunodef
- 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
- 5Hyper IgM immunodeficiency1. rare x linked 2. defect gene coding for CD40 ligand essential to class switching 3.
- 6Hyper IgM symp dx + txsymp: boys, bacterial infections. dx: def IgG and IgA, no CD40 ligand. tx: antibiotic and gammaglobulin transfusions
- 73 combined B and T cell defs1. SCID 2. ADA adenisine deanimase def 3. xlinked SCID
- 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,
- 9x linked SCIDlethal. defect in gene for gamma chain of IL-2 receptor. Need bone marrow transplant or gene tx
- 10types of acquired deficiencies1. malnutrition. 2. cancer. 3. stress. 4. exercise. 5. v young/old. 6. immunosupression 7. infections
- 11malnutritionimpaired functions due to lack of selenium copper and fe, lymphoid system particular affected, worldwide issue
- 12symptoms dx and tx of complement defssymp: recurrent bacterial infections, dx: symp + CH50 levels, tx: antibiotics
- 13glucocortcoidsinhibition of b cells, t cells and cytokines
- 14AIDS statsleading cause of death in africa. ~1 million dx in US.
- 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
- 16list of x linked immunodef diseases (5)1. CGD, 2. wiskott aldrich syndrome, 3. SCID, 4. x linked agammaglobulinaemia, 5. hyper IgM immunodef
- 17defect in CGDintracellular phagocytic killing and inability to produce bacterial cytolysins
- 18defect in wiskott aldrich syndromethrombocytopenia and low serum IgM, low CD23 expression causes abnormal B cell development
- 19defect in SCIDdefect in both humoral and cel mediated immune responses
- 20defect in Hyper IgMdefective CD40 ligand on lymphocytes prevent isotope switching therefore low serum levels of all immunoglobulin classes except IgM
- 21four components of immune systemT cells, B cells, phagocytes, complement
- 22Diagnosis of LADdetection of defect in CD11 and CD18
- 232 types immunodeficiencycongenital or acquired
- 24primary (congenital)defects in structural components or developmental stages of immune system. May be masked at birth by abs from mother.
- 25causes of secondary (acquired) immunodeficiency1. infectious disease (HIV) 2. cancer 3. stress 4. excessive exercise 5. malnutrition 6. drug tx
- 26warning signs of primary immunodef1. family hx 2. recurrent serious infections 3. infant failure to thrive 4. persistent thrush 5. need for IV antibiotics
- 27who gets repeated bacterial infections?pts with:1. low phagocyte numbers (or defective) 2. complement defects 3. humoral immunity defects
- 28defects in CMI (cell mediated immunity) usually result in recurrent......fungal protozoan intracellular bacterial and viral infections
- 29phagocytic dysfunction1 low neutrophil numbers from defective production during haemopoieses, 2. life threatening, 3. tx=recombinant granulocyte colony stimulating factrs
- 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
- 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
- 32symptoms of LAD1. recurrent bacterial infections 2. absence of neutrophils at infected sites leading to large abcesses
- 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
- 34treatment for LADantibiotics
- 35chronic granulomatous disease CGDNADPH oxidase is defective=no superoxide ions to kill intracellular bacteria
- 36what is most common cause for CGDdefect in cytochrome b
- 37what is future tx for cgdgene replacement therapy
- 38Dx of CGDneutrophils stained with nitro-blue tetrazolium remain pale yellow rather than blue
- 39Tx of CGDantibiotics
- 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
- 41three diseases associated with defective intracellular killing of ingested bacteria1. chronic granulomatous disease CGD 2. Chediak Higashi syndrome 3. macrophage defects in IFN signalling
- 42macrophage defects in IFNy signallingautosomal recesive, fatal unless tx with IFNy
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- Immunopathology Fichas sobre immunodeficiency, creado por lumen7 el 05/05/2013.
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