High Yield Inflammation

Front 1

RAT

Back 1

SIT

Front 2

for review

Back 2

binding factors

Front 3

adhesion mediated by

Back 3

Mediated by integrins ICAM-1 and VCAM-1

Front 4

for review

Back 4

review

Front 5

transmigration

Back 5

just review

Front 6

for phagocytosis: recognition and attachment

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Opsonins (IgG and C3) coat target

Front 7

phagocytosis o2 dependent killing

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O2 dep: Reactive O2 species in lysosomes

Front 8

for phagocytosis: O2 independent killing

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O2 indep: Bactericidal permeability agents, lysozyme,
MBP, lactoferrin

Front 9

for phagocytosis

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steps

Front 10

LAD 1 defect

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•B chain of CD11/CD18 integrins

Front 11

LAD 2 defect

Back 11

Sialylated oligosaccharide

Front 12

CGD defect

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defective chemotaxis:
•NADPH oxidase (membrane) - x linked
•NADPH oxidase (cytoplasm) - AR

Front 13

MPO deficiency defect

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Absent H2O2 system, cant make HCL
•no problem w infections

Front 14

Chediak-Higashi syndrome defect

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Lysosomal defect

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Thermal injury, DM, CA, sepsis acquired immunologic defect

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Chemotaxis

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Dialysis, DM - acquired immunologic defect

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Adhesion

Front 17

Leukemia, anemia, sepsis, DM,
neonates, malnutrition - acquired immunologic defect

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•Phagocytosis & microbicidal activity

Front 18

clinical findings in LAD1 (defect in CD11/CD18 integrins on polys)

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Neutrophils can roll but do not stick
• Recurrent bacterial infections
• Inflammatory lesions lack neutrophils
• But, ↑ neutrophils in the circulation

Front 19

what

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chediak-higashi: defect in lysosome so large lysosomal vesicles

Front 20

mutation in chediak-higashi

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Mutation in LYST lysosomal trafficking regulator gene

Front 21

other problems in Chediak Higashi besides defect in phagocytosis

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any cell with granules including melanosomes (ocular albinism); EBV lymphoproliferative d/o

Front 22

clinical scenario: Young, men with infections and granulomas at autopsy
– recurrent catalase + bacterial and fungal infections

think:

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CGD (xlinked, defect in NADPH oxidase system)

Front 23

Nitoblue tetrazolium reductase test negative: think

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CGD
Inability to reduce NBT
– reduced O2 consumption,
reduced H2O2 and O2- production

Front 24

deficiencies in Membrane Attack Complex (MAC C5-9), leaves you vulnerable to what:

Back 24

encapsulated
Neisseria, Strept, H. influenzae, Listeria

INSL (insulated/encapsulated)

Front 25

which complement components mediate vasodilation

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C3a and C5a

Front 26

which complement mediates activation of arach. acid pathway (lipoxygenase)

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C5a

Front 27

which complement mediates Leukocyte activation, adhesion and chemotaxis

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C5a

Front 28

which complement acts as opsonin

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C3b

Front 29

complement activation

Back 29

for review

Front 30

what is Decay accelerating factor

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acts to destabilize convertases that activate complement

Front 31

what dz occurs where defect in DAF and can't turn off complement

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PNH
you cannot turn off the complement system
-RBCs are destroyed and platelets bind to complement
-Hemolytic anemia and thrombosis

Front 32

how does AA get out of cell membrane

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phospholipases

Front 33

what are the two pathways of AA metabolism

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lipooxygenase and cyclooxygenases

Front 34

Cyclooxygenases synthesize

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Prostaglandins
Thromboxanes

Front 35

Lipoxygenases synthesize

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Leukotrienes
Lipoxins

Front 36

where steroids and asa act

Back 36

for review

Front 37

effect of TNFa and IL1

Back 37

review

Front 38

mediators released by macs

Back 38

Tox. O2
Proteases
Collagenases
Chemotx factors
Coag factors
AA metabolites
NO
PDGF
FGF
TGF

Front 39

czome for Familial retinoblastoma

Back 39

13

Front 40

NF 1 and 2 czomes

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17 and 22

Front 41

four tumors of turcot's syndrome

Back 41

(colon, breast, ovary & brain

Front 42

dna repair defects (4 syndromes)

Back 42

Xeroderma pigmentosa (XP) – 9,19,11,16,13
• Ataxia-telangiectasia – 11q22.3
• Bloom’s syndrome – 15q26.1
• Fanconi’s anemia – 9,16,3,6

Front 43

two signal transduction oncoproteins

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ras (GTP binding)
• abl (non-receptor tyrosine
kinase)

Front 44

nuclear regulatory protein oncoproteins

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myc family

Front 45

oncoproteins

Back 45

for review

Front 46

what kind of oncoprotein is her2/neu

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receptor tyrosine kinases (growth factor receptor

Front 47

for review

Back 47

read through

Front 48

how is ras gene activated

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point mutation

Front 49

aml m2 (myeloblastic with differentiation)

Back 49

t(8;21)

Front 50

aml m3 promyelocytic

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t(15;17)

Front 51

aml m4 eo myelomonocytic with eo

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inv 16

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aml m5, monoblastic

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t(9;11)

Front 53

common translocation in all

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t (4:11)

Front 54

burkitt

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t(8;14)

Front 55

follicular

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t(14;18)

Front 56

b LBL - 7 recurrent translocations

Back 56

B LBL w t(9;22)(q34;q11.2); BCR-ABL1
2. B LBL w t(v;11q23); MLL rearranged
3. B LBL w t(12;21(p13;q22);TEL-AML1
4. B LBL w hyperdiploidy
5. B LBL w hypodiploid
6. B LBL w t(5;14)(q31;q32);IL3-IGH
7. B LBL w t(1;19)(q23;p13.3)E2A-PBX1

Front 57

good b LBL genetics

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t(12;21) (p13;q22); TEL/AML1
• Hyperdiploid > 50
• Age 4-10

Front 58

bad b LBL genetics

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Hypodiploid
• t(9:22) (q34;q11.2); BCR/ABL
• 11q23; MLL
• t(1;19) (q 23;p13.3); PBX/E2A

Front 59

most common b LBL in infant

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t(4;11)

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most common b LBL translocation in child

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t(12;21)

Front 61

most common b LBL in adults

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t(9;22)

Front 62

genetics of ewings

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T(11;22) EWS/FLI-1
T(21;22) EWS/ERG
Many others EWS/Other

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genetics of DSRCT

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T(11;22) EWS/WT-1

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genetics of clear cell sarcoma

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T(12;22) EWS/ATF-1, EWS/TEC

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genetics of myxoid liposarcoma

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T(12;16) EWS/CHOP EWS/FUS

Front 66

genetics of alveolar soft part sarcoma

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T(X;17) ASPL/TFE3

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genetics of synovial sarcoma

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T(X;18) SYT/SSX1 SYT/SSX2

Front 68

genetics of rhabdomyosarcoma

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T(1;13) PAX7/FKRH
T(2;13) PAX3/FKRH

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dfsp genetics

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T(17;22) CALIA1/PDGFß

Front 70

low grade fibromyxoid sarcoma genetics

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T(7;16) FUS/CREB

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tumor suppressor genes

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Rb gene (retinoblastoma) 13q14
• p53 gene (G1 arrest apoptosis) 17p13.1
• APC gene (adenom. polyposis coli) 5q21
• NF-1 gene (neurofibromatosis) 17q11
• NF-2 gene (neurofibromatosis) 22q12
• DCC gene (deleted in colon CA) 18q21
• WT-1 gene (Wilms’ tumor) 11p13
• VHL gene (von Hippel-Lindau) 3p25

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P62

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liver, mallory bodies

Front 73

P63

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breast/prostate - myoepithelial/basal celsl

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P53

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gi tract, glandular dysplasia

Front 75

trinucleotide repeat

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Fragile X
Friedrich ataxia
Spinocerebellar ataxia
Myotonic dystrophy

Front 76

wiscott-aldrich sx

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X-linked recessive
Eczema, thrombocytopenia and immune deficiency

wiscite-aldrich where I, T, E

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arsenic carcinogenicity

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skin ca

Front 78

ebv-related cancers

Back 78

Burkitt’s Lymphoma, especially endemic
• Post-transplant lymphoproliferative disorders
• Nasopharyngeal CA
• Hodgkin lymphomas
• Primary effusion lymphoma
• Angioimmunoblastic T cell lymphoma
• Aggressive NK/T cell lymphoma

Front 79

tumors and hypoglycemia: name three

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Fibrosarcoma, solitary fibrous tumor, HCC

IGF or increased insulin

Front 80

tumor and polycythemia

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metanephric adenoma
Renal cell carcinoma
• Cerebellar hemangioblastoma
• Hepatocellular carcinoma
• Leiomyoma
• Solitary fibrous tumor

Front 81

acanthosis nigricans + tumor

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Gastric CA
• Lung CA
• Uterine CA

Front 82

tumors in nf1 and nf2

Back 82

Multiple NFs (plexiform NFs), schwannomas
(bilateral acoustics in 2), gliomas, pheos,
somatostinomas

Front 83

tuberous sclerosis tumors and genetics

Back 83

Chromosomes 9 & 16
• Cortical tubers, sclerosis, SEGAs, cardiac
rhabdomyomas, pulm LLM, AMLs, sebaceous
adenomas, ash green patches, subungual fibromas

Front 84

tumors/findings of VHL

Back 84

RCC (bilateral)
papillary cystadenoma of pancreas,
pheos
• Cavernous hemangiomas/hemangioblastomas of retina, cerebellum/brainstem
• Hemangiomas/cysts in pancreas, skin, liver,kKidney

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gardners tumors

Back 85

FAP & jaw osteomas, desmoids, cysts of skin
• Soft tissue tumors (desmoplastic fibromas)

Front 86

cowden genetics and tumors

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10q23 (same as MEN II – MEN I 11p13)
• 9q22.3
• FAP with increased risk of breast, thyroid CA
• Tricholemommas

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tumors of Peutz-jeghers

Back 87

SCTAT/Large cell calcifying Sertoli tumors
• Adenoma malignum of cervix
• Increased risk of breast, lung, ovary, pancreas and
uterine CA; rare malignant transformation in GI tract

Front 88

genetics and tumors of turcot syndrome

Back 88

AR
FAP with brain tumors (astrocytomas – GBMs)
• HNPCC genes
• DNA mismatch/repair defect

Front 89

findings of Canada-cronkhite

Back 89

FAP (hamartomatous polyps) with skin & nail changes

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findings in gorlin syndrome

Back 90

cutaneous basal cell carcinoma and
a mandible odontogenic keratocyst
Intracranial calcification
• Skeletal abnormalities: bifid ribs, kyphoscoliosis,
calcification of falx cerebri
• Distinct faces: frontal/temporopariental bossing,
hypertelorism, and mandibular prognathism

Front 91

genetics of gorlin

Back 91

Mutated PTCH gene Chr (9q)

Front 92

findings in Werner Syndrome

Back 92

MEN I, menin, czome 11 PPPAAT
Parathyroid adenomas/hyperplasia
– Pituitary adenomas/hyperplasia
– Pancreas adenomas/carcinomas/hyperplasia
– Adrenal hyperplasia (note like mini II
– Thyroid C-cell hyperplasia (note like mini II

Front 93

findings in sipple syndrome

Back 93

men II, czome 10 (ret)
Parathyroid adenomas/hyperplasia
Pheochromocytomas
Thyroid medullary carcinomas

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menIIb

Back 94

menIIa +with mucocutaneous ganglioneuromas and
marfanoid habitus (RET (different locus)

Front 95

minimal change dz in kidney

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hodgkin lymphoma

Front 96

reye

Back 96

abnl mitochondria

Front 97

assoc with Plummer Vinson

Back 97

FeDA,
pulmonary
hemorrhage

Front 98

assoc with multicentric castlemans

Back 98

POEMS

Front 99

assoc with Heinz bodies

Back 99

G6PD, unstable
HB pyruvate
kinase, sulf Hb

Front 100

mass in the lower
extremity of a female
adolescent or young
female adult and rhomboid crystals on EM

Back 100

alveolar soft parts sarcoma

Front 101

calc of Hct =

Back 101

mcv * rbc (femtoliters)

Front 102

calc of MCH

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= hb*10/rbc (picograms)

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calc of • MCHC =

Back 103

hb/hct (g/dL)