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74 Cards in this Set
- Front
- Back
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features of hypogammaglobulinemia?
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sinopulmonary infxs. due to encapsulated bacteria, conjunctivitis, dermatitis and malabsorption
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X- linked hypogammaglobulinemia?
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reduced IG's, no circulating B or plasma cells, pre B cells found, normal T cells-due to failure of VH gene translocation in pre-B cells by tyrosine kinase-chronic lung disease
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physiologic hypogammaglobulinemia?
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b/w 4-6 months where infants have low IgG, found in premature infant(maternal IgG transferred primarily in third trimester
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transient hypogammaglobulinemia?
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onset 5-6 months, often asymptomatic but may have respiratory infx.-low IgG with normal IgM and IgA, circulating B cells-may be lack of CD4 cells or maternal Ab's against fetal determinants
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common variable immunodeficiency?
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acquired hypogammaglobulinemia, onset in later life, autoimmune disorders and respiratory infx., enlarged lymph nodes and spleen, low total Ig's, B cells present but no Ab response-either suppressor T cells or B cell abnormalities
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selective IgA deficiency?
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most common immunodeficiency, increased allergies and asthma due to high IgE and autoimmune diseases. IgA low, all others normal with T cell immunity normal-block in B cell release of IgA-do not give gamma globulin(anaphylaxis)
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IgG subclass deficiency?
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recurrent infx., isolated or associated deficiencies-can't just measure total IgG
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selective IgM deficiency?
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autosomal recessive, normal B cells unable to develop to plasma cells-meningitis and severe infx.
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X-linked hyper IgM syndrome?
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IgG, IgA, IgE deficiency with increased IgM. no class switching due to mutation on CD40 ligand of T cells
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screening tests for B cell function?
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quantitative Ig's
isohemagglutinin titer IgG subclasses |
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definitive tests for B cell function?
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B cell quantitation-immunofluorescence
specific Ab response SPE or immunoelectrophoresis in vitro Ig synthesis |
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humoral immunity evaluation?
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flow cytometry for CD19(B cells)
SPE and IFE for plasma gammaglobulins nephelometry or RID for G,A,M or RIA for E ELISA for Ab's pre and post immunization |
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cellular immunity evaluation?
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CBC for total lymphocytes
flow cytometry with CD3 marker(T), CD4 or CD8 mitogen, Ag or allogenic cells response for T cell fx. candida,mumps or strep for specific cellular immunity in vivo |
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screening for T cell function?
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total lymphocyte count->1200
delayed hypersensitivity skin tests-response after 48 hours |
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definitive T cell tests?
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T cell quantitation-Ab's against CD3, CD4,CD8
response to mitogens, Ag and allogenic cells in vitro mix for helper T cell fx. |
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severe combined immunodeficiency?
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no lymph nodes or tonsils, lesions, no T or B cells, no lymphocyte response, low IgG, no IgA and normal IgM, pneumonia and fluid in lungs,
1.x-linked 2.autosomal recessive- 3.adenosine deaminase deficiency leading to toxic metabolites and lymph. inhibition 4. bare lymphocyte syndrome- MHC II deficiency usually die at 12-24 months |
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DiGeorge syndrome?
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on infx. within first 2 weeks-congenital malformation due to arrested dev. of 3rd and 4th pharyngeal pouches during weeks 6-12-thymic hypoplasia, hypoparathyroidism, aortic arch anomalies and abnormal face(fish mouth) with MR-low lymph count, low T cells and response-zinc deficiency?
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chronic mucocutaneous candidiasis?
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T cell immunity defect against Candida, assoc. with endocrine problems( Addison's, hypopara/thyroid)
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Wiskott-Aldrich syndrome?
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x-linked, bleeding due to decrease in platelet number and size due to bacteria, 5-6 month onset-high E and A with low M and normal G, no Ab response to polysaccharide Ag, start with normal T cells but deteriorate, poor prognosis-defect in WAS protein(cell signaling and cytoskeleton)- bone marrow transplant
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graft versus host disease?
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rashes, jaundice, hair loss, infx.-reduced by eliminating lymphocytes
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ataxia-telangiectasia?
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unsteady gait at 1-2 years, tortuous blood vessels in skin or sclera-no IgA, low IgM, low T cells, PHA response normal but PWM low, autosomal recessive, defective DNA repair-due to defect in homologue of phospho inositol 3 kinase(signaling)
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Nezelof syndrome?
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reduce T cell immunity with disregulation of Ig's-infx., enlarged lymph nodes, liver and spleen
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immunodeficiency with short-limbed dwarfism?
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normal head with short extremities, thin sparse hair-T,B cell and combined all found
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immunodeficiency with Thymoma?
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recurrent infx., considered in patients with hypogammaglobulinemia
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what type of reaction is an allergy?
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type I hypersensitivity
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type I hypersensitivity?
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interaction of Ag and IgE on mast cells and basophils, within minutes
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type II hypersensitivity?
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binding of IgG or IgM to cell surface Ag's-
cytotoxic-complement or phagocytic activation non-cytotoxic- destruction of ACh/thyroid receptors, hormone mimicry 5-8 hours |
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type III hypersensitivity?
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"immune complex disease"
binding of IgG or IgM to soluble Ag to form immune complexes-activate complement(C3a and C5a) then neutrophils and macro's, localized to site of accumulation, basis for autoimmune diseases, 5-8 hours |
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type IV hypersensitivity?
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"delayed type sensitivity"
Ab independent-Ag activates CD4 and CD8 cells with release of IL-2 and IF-gamma(macro activation) agents include haptens, nickel, cosmetics, poison oak, 24-72 hours(contact dermatitis and tuberculin tests) |
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urticaria/angioedema
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allergic, immunologic or non-immunologic-from foods, drugs, inhalents, cold, pressure-histamine release, edema, vascular inflammation-treat with antihistamines, corticosteroids
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urticaria
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itching with edema of superficial dermis, lesions of less than 24 hours and no marks
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angioedema
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swelling of lower dermis and subQ tissue without itching
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hereditary angioedema
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autosomal dominant-absence(type I) or dysfunction(typeII) of CI inhibitor protein-angioedema without urticaria due to complement activation and bradykinins-C2 affected
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acquired C1 inhibitor deficiency
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autoAb against C1INH, low C1-secondary to tumors and conn. tissue disorders
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allergic contact dermatitis?
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type IV reaction with a cell-mediated response-CD4 cells-langerhans cells present Ag to T cells with release of cytokines-direct area of exposure involved with redness and swelling
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irritant contact dermatitis?
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does not involve Ag-specific mechanisms-CD8
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Patch testing?
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used to ID specific allergens
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atopic dermatitis?
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itching with lesions, high IgE and defective cell-mediated immunity-treated with agents to block T cell function via inhibition of IL-2 transcription
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hypersensitivity vasculitis?
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abnormal systemic immune response to drugs or bacteria with complex deposition in postcapillary venules-palpules on lower extremities, up to four weeks with scars-necrosis of venules by PMN's
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Henoch-Schonlein Pupura?
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superficial vasculitis with IgA deposition following an upper respiratory infx.
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Pemphigus?
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"intra epidermal blistering disease"
cytotoxic type II-autoAb against adhesion molecules leading to dermal separation 1.pemphigus vulgaris-IgG autoAb to desmoglein 1 or 3 2. bullous pemphigoid-IgG autoAb to hemidesmosome Ag |
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Stevens-Johnson syndrome/toxic epidermal necrolysis?
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mucocutaneous hypersensitivity rxn due to response in skin to drugs or metabolites-widespread necrosis and epidermal detachment resembling scalding-complexes and CD 8 cells-treat like burn patient
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hypersensitivity pneumonitis?
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interstitial or alveolar granulomatous disease due to organic dust inhalation-restrictive(air can't cross the alveolar border)-in males 30-50, cough, fever, malaise-type III and IV, key is precipitating Ab(IgG) to Ag, increase in CD 8 cells but abnormal fx., high erythrocyte sedimentation
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asthma?
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obstructive(can't get air to alveoli), chronic inflammation of the airways caused by IgE-wheezing, cough, chest tightness-mast,epithelial and T cells with macro's and eosinophils-beta agonists, anticholinergics, corticosteroids
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allergic bronchopulmonary aspergillosis?
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type I and III, in pts. with asthma or CF, both hypersensitivity rxn. and immune complex disease-IgE and IgG to fungus aspergillus fumigatus without infx.-elevated IgE-type I in lung first, then complex and complement-bronchodilators
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Goodpastures syndrome?
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"antiglomerular basement membrane disease"
adult males 20-50, cough up blood and protein in urine, Ab to basement membrane Ab against type IV collagen- viral infx., IgG deposits and complement bound to alveolie |
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Wegener's granulomatosis?
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type II,III and IV-affects respiratory tract and kidneys associated with antineutrophil autoAb's(serine protease 3), males 40-50, nasal complaints, cough, ulcerations-
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Sarcoidosis
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systemic granulomatous with unknown origin, restrictive affecting AA, Puerto Ricans and Scandinavians 30-40-weight loss, fatigue, cough, bloody sputum, skin lesions-type IV with increased CD 4
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hemolytic disease of the newborn?
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"erythroblastosis fetalis"
type II cytotoxic-placental IgG for an RBC Ag, problem with Rh- mom and Rh+ SECOND child, T dependent-treated with RhoGAM at 28 weeks and 24 hours post birth(eliminates fetal Rh+ cells from child) |
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myasthenia gravis?
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autoAb to ACh receptors on post-synaptic membrane-internalization and destruction-ptosis, diplopia, dysarthria, dysphagia, respiratory problems
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Grave's disease?
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autoAb against TSH receptor in thyroid stimulating the receptor and increasing TH
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Hashimoto's thyroiditis?
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autoAb to thyroglobulin and thyroperoxidase(no role in pathogenesis of the disease)- become hypothyroid via type IV
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hypersensitivity pneumonitis?
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interstitial or alveolar granulomatous disease due to organic dust inhalation-restrictive(air can't cross the alveolar border)-in males 30-50, cough, fever, malaise-type III and IV, key is precipitating Ab(IgG) to Ag, increase in CD 8 cells but abnormal fx., high erythrocyte sedimentation
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asthma?
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obstructive(can't get air to alveoli), chronic inflammation of the airways caused by IgE-wheezing, cough, chest tightness-mast,epithelial and T cells with macro's and eosinophils-beta agonists, anticholinergics, corticosteroids
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allergic bronchopulmonary aspergillosis?
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type I and III, in pts. with asthma or CF, both hypersensitivity rxn. and immune complex disease-IgE and IgG to fungus aspergillus fumigatus without infx.-elevated IgE-type I in lung first, then complex and complement-bronchodilators
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Goodpastures syndrome?
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"antiglomerular basement membrane disease"
adult males 20-50, cough up blood and protein in urine, Ab to basement membrane Ab against type IV collagen- viral infx., IgG deposits and complement bound to alveolie |
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Wegener's granulomatosis?
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type II,III and IV-affects respiratory tract and kidneys associated with antineutrophil autoAb's(serine protease 3), males 40-50, nasal complaints, cough, ulcerations-
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Sarcoidosis
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systemic granulomatous with unknown origin, restrictive affecting AA, Puerto Ricans and Scandinavians 30-40-weight loss, fatigue, cough, bloody sputum, skin lesions-type IV with increased CD 4
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hemolytic disease of the newborn?
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"erythroblastosis fetalis"
type II cytotoxic-placental IgG for an RBC Ag, problem with Rh- mom and Rh+ SECOND child, T dependent-treated with RhoGAM at 28 weeks and 24 hours post birth(eliminates fetal Rh+ cells from child) |
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myasthenia gravis?
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autoAb to ACh receptors on post-synaptic membrane-internalization and destruction-ptosis, diplopia, dysarthria, dysphagia, respiratory problems
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Grave's disease?
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autoAb against TSH receptor in thyroid stimulating the receptor and increasing TH
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Hashimoto's thyroiditis?
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autoAb to thyroglobulin and thyroperoxidase(no role in pathogenesis of the disease)- become hypothyroid via type IV
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systemic lupus erythematosus?
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type III autoimmune, females 15-45-estrogen influence-Ab both localized and systemic in nuclei-table of symptoms page 279
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type I diabetes
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type IV autoimmune disease
autoAb against islet cells and pancreas but minor role rather Ag specific T cell activation and lymphocyte infiltration to pancreas |
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C5-8 or properdin deficiency
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Neisserial infection, meningitis
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C1q, C2 or C3 deficiency
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infection from encapsulated bacteria
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C1, C4 or C2 deficiency
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immune complex disease
dermatitis, lupus, arthritis |
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C1 inhibitor deficiency
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dominant
low levels of C4 and C2 angioedema C3-C9 unaffected treat with steroids |
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C3 regulatory Factor I and H deficiency
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bacterial sepsis and glomerulo-nephropathy
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leukocyte adhesion deficiency
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complement receptor deficiency
ulcerating infections, extracellular bacterial infection and abnormal wound healing |
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chronic granulomatous disease
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NADPH oxidase deficiency-no superoxide anion
catalase positive infections-staph,serratia,aspergillus,klebsiella pneumonia |
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leukocyte glucose 6 phosphate DH deficiency
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catalase positive infections-staph, serratia, aspergillus, klebsiella pneumonia
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myeloperoxidase deficiency
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mild or no symptoms
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Chediak-Higashi syndrome
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only disease with lack of NK function,
partial albinism, recurrent bacterial infection |
