6 Path 230-255

Front 1

x-linked agammaglobulinemia?

Back 1

failure of b-cell precursors to develop and mature into mature B cells

Front 2

mutation in x-linked agammaglobulinemia

Back 2

bruton tyrosine kinase (Btk) - associated with Ig receptor complex of pre-B and when mutated cannot deliver signals, and maturation stops

Front 3

infections common in x-linked agammaglobulinemia

Back 3

bac - Haem influ, strep pneu, staph.A,
vir- echovirus, poliovirus
giardia lamblia

Front 4

x-linked agammaglobulinemia, when and who

Back 4

presents in males at 6 months (when mother's immunoglobulins run out)

Front 5

common variable immunodeficiency, who when

Back 5

m and f. childhood

Front 6

common variable immunodeficiency what

Back 6

deficiency in antibodies

Front 7

histo common variable immunodeficiency

Back 7

b cell areas are hyperplastic, due to lack of regulation because there are no antibodies to stop growth when antigen is presented

Front 8

isolated IgA deficiency causes problems with what?

Back 8

mucosal protection. GI, pulmonary, GU

Front 9

hyper-IgM syndrome, what?

Back 9

mutation in CD40 which is responsible for class switching of Ig. So IgM, but no IgG, IgA, or IgE.

Front 10

hyper-IgM syndrome, complications?

Back 10

IgM can attack rbcs, hemolytic anemia, thrombocytopenia, neutropenia. pneumocystis jiroveci

Front 11

Di george?

Back 11

T cell deficiency due to failure of development of 3rd and 4th pharyngeal pouches. Lack of thymus, parathyroid, parts of thyroid. loss of T cells, tetany, heart and vessel defects

Front 12

severe combined immunodeficiency, presents with?

Back 12

thrush, diaper rash, failure to thrive

Front 13

severe combined immunodeficiency affects what?

Back 13

both humoral and cell immunity

Front 14

severe combined immunodeficiency types and genes

Back 14

x linked - gamma-chain unit of cytokine receptors. IL-7 required for t cell development. 15 for NK cells

autosomal recessive - deficiency in adenosine deamineas (ADA). accumulation of ADA and dmg to immature lymphocytes

Front 15

wiskott-aldrich syndrome presents with?

Back 15

thrombocytopenia, eczema, marked vulnerability to recurrent infection, ending in early death

Front 16

deficiency in C2 causes

Back 16

asymptomatic as alt pathwar can compensate, but increased risk of SLE

Front 17

deficiency in C5, 6. 7 8. 9 cause?

Back 17

inability to make MAC. sus to neisseria

Front 18

deficiency in C1 inhibitor

Back 18

hereditary angioedema

Front 19

3 major routes for HIV

Back 19

parenteral inoc, sexual, mothers to newborns

Front 20

structure of HIV

Back 20

core: capsid protein p24, 2 copies of RNA, nucleocapsid protein p7/p9, 3 viral enzymes (proteases, reverse transcriptase, integrase)

core surrounded by matrix protein p17

viral envelope with gp120 and gp41

Front 21

hiv genes

Back 21

gag, pol, env

gag and pol targets of anti-HIV-1 protease inhibitor drugs

Front 22

hiv infection process - steps

Back 22

gp120 binds to CD4, conformational change in gp120 so it can bind to chemokine receptors CCR5 or CXCR4, conformational change in gp41 so it can bind to membrane and virus can be internalized

Front 23

hiv completion of life cycle of latently infected cells how?

Back 23

only after cell activation, as by antigen or cytokines that upregulate transcription factors such as NF-kappaB

Front 24

selective loss in which type of cell in early HIV that explains poor recall responses to previously encountered antigens

Back 24

CD4+ helper T cells

Front 25

effect of HIV on macrophages?

Back 25

infects them but doesnt affect function, but they can serve as reservoirs and spread disease

Front 26

effect or HIV on dendritic cells

Back 26

mucosal dendritic cells are infected and transport it to regional lymph nodes. follicular dendritic cells in lymph nodes serve as reservoirs

Front 27

effect of HIV on B cells

Back 27

B cell germinal hyperplasia, bone marrow plasmacytosis, hpergammaglobulinemia, formation of circulating immune complexes.

Front 28

cells damaged in CNS HIV

Back 28

macrophages and mircoglial cells

Front 29

? are the largest reservoirs of T cells in the body

Back 29

mucosal tissues

Front 30

? are sites of continuous HIV replication and cell destruction

Back 30

lymph nodes and spleen

Front 31

most common fungal in aids

Back 31

candidiasis. transformation to oral cand heralds the transition to aids

Front 32

CMV and HIV

Back 32

less than 50 CD4. eye and GI tract most commonly

Front 33

HIV common bacteria

Back 33

atypical mycobacteria and TB

Front 34

common cancers in HIV

Back 34

KS, non hodgkin B cell lymphoma, cervical cancer women and anal cancer men

Front 35

what is KS?

Back 35

vascular tumor, proliferation of spindle-shaped cells that express markers for endothelial cells and smooth muscle cells.

Front 36

method of KS infection

Back 36

actually in infection by KSHV. but KSHV cannot cause KS on its own, needs cofactor. HIV provides that cofactor

Front 37

3 types of hiv related lymphomas

Back 37

systemic (lymph nodes), primary CNS, body-cavity based

Front 38

how to lymphomas form in HIV?

Back 38

sustained polyclonal B cell activation, leading to mutations

Front 39

what is amyloidosis?

Back 39

pathological proteinaceous substance, deposited in the extracellular space in various tissues and organs of the body in a wide variety of clinical settings. results from abnormal folding of proteins, which are deposited as fibrils in extracellular tissues and disrupt normal function

Front 40

histo for amyloidosis?

Back 40

congo red stain, it stains green

Front 41

AL form of amyloidosis. stimulus, affected cells, soluble precursor, insoluble fibrils

Back 41

unknown (carcinogen?), monoclonal B-lymphocyte proliferation of plasma cells, immunoglobulin light chains, AL protein

Front 42

AA form of amyloidosis. stimulus, affected cells, soluble precursor, insoluble fibrils

Back 42

chronic inflammation and macrophage activation, liver cells, SAA protein, AA protein

Front 43

Beta-amyloid (A-beta) protein found in?

Back 43

alzheimer's

Front 44

pathogenesis of amyloidosis

Back 44

not due to overproduction of AA, AL...but lack of ability to break down the amyloid. lack of the enzymes necessary. or due to abnormality that makes saa... resistant to degradation

Front 45

AL associated diseases

Back 45

multiple myeloma

Front 46

what are bence-jones proteins?

Back 46

light chains of Ig molecules. seen in AL and multiple myeloma

Front 47

primary amyloidosis, imunocyte dyscrasias with amyloidosis

Back 47

AL

Front 48

reactive systemic amyloidosis

Back 48

AA

Front 49

reactive systemic amyloidosis complicates what other diseases?

Back 49

rheumatoid arthritis, ankylosing spondylitis, inflammatory bowel disease

Front 50

hemodialysis associated amyloidosis

Back 50

Beta2- microglobulin

Front 51

amyloid of aging, affects what tissues primarily?

Back 51

TTR, heart

Front 52

sago spleen

Back 52

deposits of amyloid largely limited to the splenic follicles, producing tapioca-like granules

Front 53

lardaceous spleen

Back 53

amyloid involves the walls of the splenic sinuses and CT framework in the red pulp

Front 54

AIDS meningitis

Back 54

cryptococcosis

Front 55

invades CNS in AIDS - enchephalitis

Back 55

toxoplasma gondii

Front 56

AIDS - diarrhea

Back 56

cruptosporidium, isopora belli. or salmonella shig, M avium