Ward Prep: Brs Ch 5 Immune Dysfunction

Front 1

What is the normal ratio of CD4 to CD8 T cells?

Back 1

2:1 CD4:CD8. Decreases w/ HIV infection

Front 2

Name the 3 complement pathways, what initiates them, and 3 outputs

Back 2

Classical pathway (Ag-Ab complex), Alternate pathway (surface of pathogens), Mannose Binding Lectin (lectin on microbial surfaces). Inflammation/thrombosis(?), Opsonization, Cell lysis (MAC)

Front 3

What are the 3 human leukocyte antigen class I antigens

Back 3

HLA-A, -B, -C

Front 4

What are the 3 human leukocyte antigen class II antigens

Back 4

HLA-DP, -DQ, -DR

Front 5

Which HLA antigens are primarily used for determining the probablity of tissue graft rejection

Back 5

HLA-A, HLA-B

Front 6

Which HLA antigen is a/w anklosing spondylitis

Back 6

HLA-B27

Front 7

Distinguish btwn the 4 types of hypersensitivity reactions

Back 7

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

Front 8

What are the 3 types of transplant rejection? Distinguish btwn their timescales and pathogenesises

Back 8

(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).

Front 9

GVH: pathophys and px

Back 9

transplanted CD8T directly damage host. CD4T recruit Mph => damage host. Fever, rash, hepatosplenomegaly, jaundice. (usu organs: skin, liver, GI mucosa)

Front 10

Tx for acute rejection?

Back 10

cyclosporin and OKT3 (anti-CD3 ab… T cells)

Front 11

Name 7 immunodeficiency dz

Back 11

X-linked agammaglobulinemia of Bruton, Wiskott-Aldrich Syndrome, Severe Combined Immunodeficiency Disease, Isolated IgA Deficiency, Common variable immunodeficiency, DiGeorge Syndrome, AIDS

Front 12

X-linked agammaglobulnemia of Bruton. Defect? When px? Manifestations?

Back 12

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)

Front 13

Isolated IgA deficiency. Sx?

Back 13

Asx or recurrent mucosal infections (URI, diarrhea). A/w anaphylaxis to transfused blood

Front 14

Common variable immunodeficiency. Which cells are affected? Px?

Back 14

Diverse disorders of B cell maturation => hypoimmunoglobulinemia => recurrent bacterial infections

Front 15

DiGeorge syndrome. Manifestations? Embryological defect?

Back 15

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

Front 16

Severe Combined Immunodeficiency Disease. Defect? Px?

Back 16

Adenine deaminase (ADA) => B and T cell deficienc. Severe infections, cancer, failure to thrive, GVH from blood transfusions. Tx: bone marrow tplant

Front 17

Wiskott Aldrich syndrome. Manifestations?

Back 17

1) eczema (red papulovesicular rash => scales) 2) thrombocytopenia 3) recurrent infections. Poor ab response to polysaccharide ag

Front 18

AIDS. 3 stages: characteristics?

Back 18

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

Front 19

AIDS opportunistic infections affecting the brain

Back 19

CMV encephalopathy, cryptococcal meningitis, toxoplasmosis, PML (progressive multifocal leukoencephalopathy, jc virus), AIDS dementia

Front 20

AIDS opportunistic infections affecting the eyes

Back 20

CMV retinitis

Front 21

AIDS opportunistic infections affecting the oropharynx, esophagus

Back 21

CMV esophagitis, HSV, esophagitis, candida, EBV oral hairy leukoplakia

Front 22

AIDS opportunistic infections affecting the lungs

Back 22

Pneumocystis carnii pneumonia, TB, histoplasmosis

Front 23

AIDS opportunistic infections affecting the gi

Back 23

CMV colitis, MAI complex, cryptosporidosis

Front 24

AIDS opportunistic infections affecting the skin

Back 24

Kaposi sarcoma (HHV8), shingles (VZV)

Front 25

AIDS opportunistic infections affecting the genitals

Back 25

HSV, HPV (warts and cervical cancer)

Front 26

What cancers to AIDS patients get

Back 26

Kaposi sarcoma and B-cell non-Hodgkin lymphoma (also, hodgkin, and hcc)

Front 27

Name 7 conective tissue disease

Back 27

fibrinoid necrosis of connective tissue. Systemic Lupus Erythematosus, Scleroderma/CREST, Sjogren syndrome, Mixed Connective tissue disease, Polymyositis/Dermatomyositis, Polymyalgia rheumatica, Polyarteritis nodosa

Front 28

Mainfestations of systemic lupus erythematosus

Back 28

"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

Front 29

Progressive Systemic Sclerosis = Scleroderma. Manifestations? Abs?

Back 29

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

Front 30

Which auto antibodies are associated with systemic lupus erythematosus

Back 30

Anti-dsDNA, anti-Smith, anti-PL (e.g. cardiolipin)

Front 31

What is CREST syndrome

Back 31

Limited variant of Scleroderma. A/w both Anti-Scl70 AND Anti-centromere. Calcinosis, Raynaud, Esophageal dysfunc, Sclerodactyly, Telangiectasia

Front 32

Sjogren syndrome. Triad of manifestations? A/w what tumor? Abs?

Back 32

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

Front 33

What is sicca syndrome

Back 33

Keratoconjunctivitis sicca and xerostomia. Sjogren's w/o CT dz

Front 34

polymyositis/dermatomyositis. Manifestations? Px? Labs? Dx?

Back 34

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)

Front 35

Polymyalgia rheumatica. Manifestatons? A/w

Back 35

muscle pain and stiffness, but NO weakness. A/w temporal (giant cell) arteritis

Front 36

Mixed connective tissue disease. Abs?

Back 36

shares clinical fx w/ other CT dz. Anti-nRNP.

Front 37

Polyarteritis nodosa. Type of immune rxn? Type of vessels? Mostly men or women?

Back 37

IC complex vasculitis. Small/medium arteries. Mostly men.

Front 38

1° amyloidosis. What are the fibrils derived from? A/w?

Back 38

immunocytic dyscrasia amyloidosis. AL = amyloid light chain = Ig light chains. A/w plasma cell disorders… surprise! (multiple myeloma, waldenstrom macroglobulinemia)

Front 39

2° amyloidosis. What are the fibrils derived from? What causes it?

Back 39

AA <= serum amyloid associated protein <= chronic tissue destruction/inflammation (RA, TB, osteomyelitis, syphilis, leprosy). Also a/w RCC, Hodkins dz

Front 40

Senile amyloidosis. Clinical sig?

Back 40

Transthyretin (serum prot that TRANSports THYroxine and RETINol) -derived amyloid accumulates. Incidental finding on autopsy

Front 41

Alzheimer disease. What's the amyloid made from?

Back 41

amyloid beta protein

Front 42

name 4 disorders a/w amyloid deposits

Back 42

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)