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42 Cards in this Set

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What is the normal ratio of CD4 to CD8 T cells?
2:1 CD4:CD8. Decreases w/ HIV infection
Name the 3 complement pathways, what initiates them, and 3 outputs
Classical pathway (Ag-Ab complex), Alternate pathway (surface of pathogens), Mannose Binding Lectin (lectin on microbial surfaces). Inflammation/thrombosis(?), Opsonization, Cell lysis (MAC)
What are the 3 human leukocyte antigen class I antigens
HLA-A, -B, -C
What are the 3 human leukocyte antigen class II antigens
HLA-DP, -DQ, -DR
Which HLA antigens are primarily used for determining the probablity of tissue graft rejection
HLA-A, HLA-B
Which HLA antigen is a/w anklosing spondylitis
HLA-B27
Distinguish btwn the 4 types of hypersensitivity reactions
Type 1: Immediate/anaphylactic; 1st exposue IgE prod by plasma cells binds FcR on mast and basophils, 2nd exp ag binds IgE, xlinks, histamine, other factors rel acutely (vasoD, bronchoC) and eosinophilia/chronic infl over time. E.g. asthma, hay fever, angioedema but not Hereditary A. Type 2: ab-mediated, ag bound to cell/ecm; (1) complement fixing ab => lysis; eg erythroblastosis fetalis, goodpasture. (2) ADCC. Fc => NK => death. (3) activating ab: Graves dz. Type 3: IC (free ag): => complement => nphil => vessel damage => thrombosis. E.g. SLE, PAN. Type 4: Cell-mediated; (1) delayed hypersensitivity: 1st exp Mph => CD4T=> memory, 2nd exp memory activate mph phago; e.g. tuberculin rxn, contact dermatitis. (2) CTL-med: tumor, virus-infected
What are the 3 types of transplant rejection? Distinguish btwn their timescales and pathogenesises
(1) Hyperacute: mins (preformed abs => localized necrosis/thrombosis). (2) Acute: days-wks (mostly T, mphage). (3) Chronic: mo-yr (abs => vasc dam => fibrosis. E.g shrunken kidney).
GVH: pathophys and px
transplanted CD8T directly damage host. CD4T recruit Mph => damage host. Fever, rash, hepatosplenomegaly, jaundice. (usu organs: skin, liver, GI mucosa)
Tx for acute rejection?
cyclosporin and OKT3 (anti-CD3 ab… T cells)
Name 7 immunodeficiency dz
X-linked agammaglobulinemia of Bruton, Wiskott-Aldrich Syndrome, Severe Combined Immunodeficiency Disease, Isolated IgA Deficiency, Common variable immunodeficiency, DiGeorge Syndrome, AIDS
X-linked agammaglobulnemia of Bruton. Defect? When px? Manifestations?
BTK (B cell tyrosine kinase) => defect in B cell development => no pasma cells, no immunoglobulins. Px after 6 mo (maternal abs). Sx: recurrent bacterial infections (strep, staph, haemophilus influenzae)
Isolated IgA deficiency. Sx?
Asx or recurrent mucosal infections (URI, diarrhea). A/w anaphylaxis to transfused blood
Common variable immunodeficiency. Which cells are affected? Px?
Diverse disorders of B cell maturation => hypoimmunoglobulinemia => recurrent bacterial infections
DiGeorge syndrome. Manifestations? Embryological defect?
CATCH22. Cardiac (congenital heart disease, Abnormal faces, Thymic hypoplasia (T cell deficit => viral and fungal inf), Cleft palate, Hypoparathyroidism (hypocalcemia => tetany). Aberrant development of 3rd 4th embyronic arch
Severe Combined Immunodeficiency Disease. Defect? Px?
Adenine deaminase (ADA) => B and T cell deficienc. Severe infections, cancer, failure to thrive, GVH from blood transfusions. Tx: bone marrow tplant
Wiskott Aldrich syndrome. Manifestations?
1) eczema (red papulovesicular rash => scales) 2) thrombocytopenia 3) recurrent infections. Poor ab response to polysaccharide ag
AIDS. 3 stages: characteristics?
Acute Retroviral Syndrome: ~1mo post infection, non-specific, flu-lke sx. Middle Chronic Phase: viral replication, Asx or lymphadenopathy, VZV reactivation, candida. AIDS: fever, fatigue, weightloss, opportunistic infections, 2° malignancies, neurological
AIDS opportunistic infections affecting the brain
CMV encephalopathy, cryptococcal meningitis, toxoplasmosis, PML (progressive multifocal leukoencephalopathy, jc virus), AIDS dementia
AIDS opportunistic infections affecting the eyes
CMV retinitis
AIDS opportunistic infections affecting the oropharynx, esophagus
CMV esophagitis, HSV, esophagitis, candida, EBV oral hairy leukoplakia
AIDS opportunistic infections affecting the lungs
Pneumocystis carnii pneumonia, TB, histoplasmosis
AIDS opportunistic infections affecting the gi
CMV colitis, MAI complex, cryptosporidosis
AIDS opportunistic infections affecting the skin
Kaposi sarcoma (HHV8), shingles (VZV)
AIDS opportunistic infections affecting the genitals
HSV, HPV (warts and cervical cancer)
What cancers to AIDS patients get
Kaposi sarcoma and B-cell non-Hodgkin lymphoma (also, hodgkin, and hcc)
Name 7 conective tissue disease
fibrinoid necrosis of connective tissue. Systemic Lupus Erythematosus, Scleroderma/CREST, Sjogren syndrome, Mixed Connective tissue disease, Polymyositis/Dermatomyositis, Polymyalgia rheumatica, Polyarteritis nodosa
Mainfestations of systemic lupus erythematosus
"I'M DAMN SHARPER" Ig (anti-Sm, -dsDNA, -PL [cardiolipin]), Malar rash, Discoid rash, Antinuc abs (see above), Mucositis (oropharyngeal ulcers), Neuro, Serositis (pleuritis, pericarditis), Hem (vasculitis), Arthritis, Photosensitivity, Renal (glom neph), Endocarditis (Libman-Sacks), Raynaud phenom
Progressive Systemic Sclerosis = Scleroderma. Manifestations? Abs?
systemic fibrosis. (px: fixed facial appearance, sclerodactyly, raynaud, esophageal dysfunc, interstitial pulmonary fibrosis, htxn). Anti-Scl-70. If also anti-centromere, then CREST syndrome: Calcinosis, Raynaud, Esophageal dysfunction, Sclerodactyly, Telangiectasia. CREST= limited version
Which auto antibodies are associated with systemic lupus erythematosus
Anti-dsDNA, anti-Smith, anti-PL (e.g. cardiolipin)
What is CREST syndrome
Limited variant of Scleroderma. A/w both Anti-Scl70 AND Anti-centromere. Calcinosis, Raynaud, Esophageal dysfunc, Sclerodactyly, Telangiectasia
Sjogren syndrome. Triad of manifestations? A/w what tumor? Abs?
1) keratoconjunctivitis sicca (dry eyes). 2) xerostomia (dry mouth) 3) a connective tissue disorder. A/w MALToma of parotid gland. Anti-SS-B. If only keratoconjunctivitis sicca + xerostomia then = sicca syndrome
What is sicca syndrome
Keratoconjunctivitis sicca and xerostomia. Sjogren's w/o CT dz
polymyositis/dermatomyositis. Manifestations? Px? Labs? Dx?
Muscle weakness starting w/ proximal. Dermato: + Heliotrope rash on face+neck. Difficulty sitting up (esp toilet). A/w malignancies. Labs: inc creatine kinase. Dx: muscle biopsy (inflam, nec)
Polymyalgia rheumatica. Manifestatons? A/w
muscle pain and stiffness, but NO weakness. A/w temporal (giant cell) arteritis
Mixed connective tissue disease. Abs?
shares clinical fx w/ other CT dz. Anti-nRNP.
Polyarteritis nodosa. Type of immune rxn? Type of vessels? Mostly men or women?
IC complex vasculitis. Small/medium arteries. Mostly men.
1° amyloidosis. What are the fibrils derived from? A/w?
immunocytic dyscrasia amyloidosis. AL = amyloid light chain = Ig light chains. A/w plasma cell disorders… surprise! (multiple myeloma, waldenstrom macroglobulinemia)
2° amyloidosis. What are the fibrils derived from? What causes it?
AA <= serum amyloid associated protein <= chronic tissue destruction/inflammation (RA, TB, osteomyelitis, syphilis, leprosy). Also a/w RCC, Hodkins dz
Senile amyloidosis. Clinical sig?
Transthyretin (serum prot that TRANSports THYroxine and RETINol) -derived amyloid accumulates. Incidental finding on autopsy
Alzheimer disease. What's the amyloid made from?
amyloid beta protein
name 4 disorders a/w amyloid deposits
Familial mediterranean fever (AA, wide distibution), medullary carcinoma of thyroid (calcitonin, w/in tumor), diabetes type 2 (in islet cells, amylin=islet amyloid), dialysis-associated amyloidosis (joints, from beta-microglobulin which is not easily filtured by dialysis)