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STEP1 > Pathology + Hem/Onc > Flashcards

Pathology + Hem/Onc Flashcards

1
Q

what is the exception where metaplasia of this does not lead to increased risk of cancer?

A

apocrine metaplasia of breast

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2
Q

vitamin ____ deficiency can lead to metaplasia

specifical example: ______

A

vit A

keratomalacia. in eye, thin squamous of conjunctivia -> stratified keratinizing squamous epithelium = keratomalacia

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3
Q

cyanosis with chocolate covered blood

dx and tx?

A

methemoglobinemia. too much Fe3+ compared to Fe2+

tx: methylene blue + vit C

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4
Q

coagulative necrosis happens from what type of injury?

A

ischemic infarction

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5
Q

liquefactive necrosis happens in 3 organs/scenarios

A
  1. brain infarction
  2. abscess (proteolytic enzymes from neutrophils)
  3. pancreatitis. parenchyma is liquefactive (vs peripancreatic fat which is saponification)
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6
Q

ischemia of lower limb and GI tract results in this type of necrosis

A

gangrenous necrosis (it’s a type of coagulative necrosis)

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7
Q

Saponification vs metastatic calcification

A

saponification: necrotic tissue acts as nidus for calcification when serum calcium is NORMAL

metastatic calcification: normal tissue, HIGH serum calcium or phosphate levels (happens in hyper PTH)

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8
Q

Fibrinoid necrosis happens where?

A

blood vessel wall. happens in malignant hypertension (onion skinning) or vasculitis

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9
Q

Endonucleases break down ______

A

DNA

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10
Q

Intrinsic mitochondrial apoptotic pathway: inactivation of ____ allows cytochrome c to leak from ______ of mitochondria -> activate caspases

A
  1. Bcl2

2. inner mitochdonrial matrix

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11
Q

extrinsic receptor-ligand apoptotic pathway:
FAS ligand binds FAS receptor (CD____) -> activated caspases
or
____ binds to its receptor -> activated caspases

A

CD95 = fas receptor

or TNF

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12
Q

____ from CD8+ t cells can trigger apoptosis

A

perforins create pores in cell

granzyme enters said pores and activates caspases

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13
Q

elimiation of free radicals. for each enzyme, name the rxn it does and location in cell:

  1. superoxide dismutase
  2. glutathione peroxidase
  3. catalase
A
  1. superoxide dismutase. mitochondria. superoxide O2 -> H2O2
  2. glutathione peroxidase. mitochondria. hydroxy free radical OH- -> Gs-SG and H2O
  3. catalase. peroxisomes. H2O2 -> O2 and H2O
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14
Q

CCl4 is converted to CCl3 free radical by ________, resulting in cell injury.
decreased ______ leads to fatty change in liver**

A

P450 system. hepatocytes.

apolipoproteins (bc dec protein synthesis bc cell injury)

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15
Q

2ndry structure of amyloid

A

beta pleated sheet

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16
Q

primary amyloidosis is systemic deposition of ____, derived from _____

A

AL amyloid, derived from immunoglobulin LIGHT chain

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17
Q

2ndry amyloidosis is systemic deposition of ____, derived from _____

A

AA amyloid, derived from serum amyloid associated protein (SAA) which is an acute phase reactant

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18
Q

Mediterranean man presents with episodes of fever and acute serosal inflammation (seems like appendicitis, arthritis, or MI, but it’s not). what is he at increased risk for?

A

amyloidosis, bc this condition (FMF) has high SAA -> 2ndry amyloidosis

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19
Q

what kind of cardiomyopathy does amyloidosis most commonly cause

A

restrictive.

note that it can also cause arrhythmias.

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20
Q

T or F, tongue enlargement, malabsorption, and hepatosplenomegaly are features of systemic amyloidosis

A

True

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21
Q

in senile cardiac amyloidosis, what exactly gets deposited? presentation?

A

non-mutated serum transthyretin

if does present, its >80 yrs of age usually

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22
Q

in familial amyloid cardiomyopathy what exactly gets deposited?

A

mutated serum transthyretin -> restrictive cardiomyopathy

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23
Q

how can DM type II cause amyloidosis

A

amylin is derived from insulin. deposits in pancreatic islets

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24
Q

dialysis associated amyloidosis bc dialysis is not good at getting rid of what protein?

A

B2-microglobulin

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25
Q

“tumor cells in an amyloid background” is what kind of cancer? what is deposited within the tumor (the amyloid background)?

A

medullary thyroid carcinoma

calcitonin, made by tumor cells, deposits in tumor
calcitonin will also be elevated in the serum

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26
Q

which neurons are most susceptible to hypoxic injury? (3)

A

Purkinje cells of cerebellum
pyramidal cells of hippocampus
pyramidal cells of neocortex

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27
Q

wound repair initial step?

A

granulation tissue formation. fibroblasts deposit ype III collagen

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28
Q

collagenase removes type ___ collagen in wound healing to be replaced by type ____. and requires ____ as cofactor

A

remove III to be replaced by I

zinc cofactor

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29
Q

which growth factors are important for angiogenesis

A

FGF and VEGF (and TGF-B)

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30
Q

vit C is needed for hydroxylation of ____ and _____ procollagen residues

A

proline, lysine

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31
Q

copper is a cofactor for _______ which cross links lysine and hydroxylysine to form stable collagen

A

lysyl oxidase

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32
Q

hypertrophic scars have type ____ collagen

vs keloid scars have type ________ and ____ collagen

A

hypertrophic III

keloid I and III

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33
Q

what conditions do you have decreased ESR? (5)

A

sickle cell anemia (other anemias are increased ESR)
polycythemia (apparent decrease b/c RBCs dilute)
HF
microcytosis
hypofibrinogenemia

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34
Q

arsenic exposure increases risk of what cancers? (3)

A

squamous cell of skin
lung cancer
angiosarcoma of liver

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35
Q

naphthylamine is found in what? increases risk for which cancer?

A

cigarette smoke

urothelial carcinoma of bladder

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36
Q

vinyl chloride is found in what? increases risk for which cancer?

A

PVC pipes

angiosarcoma of liver

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37
Q

ionizing radiation (nuclear and radiotherapy) increases risk for which cancers? (3) how?

A

by generating hydroxyl free radicals

  1. AML
  2. CML
  3. papillary carcinoma of thyroid
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38
Q

nonionizing radiation (sunlight, UVB) causes cancer how? what enzyme usually works against this?

A

forms pyrimidine dimers in DNA

normally excised by restriction endonuclease but is overwhelmed w/ too much radiation exposure

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39
Q

mutated Ras keeps itself activated by inhibiting _________

A

GTPase activating protein

Ras normally cleaves GTP to GDP to inactivate itself, this is augmented by GTPase activating protein

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40
Q

_________ complex phosphorylates Rb, which promotes progression through G1/S checkpoint by _______

A

cyclinD/CDK4 complex phosphorylates Rb -> phosphorylated Rb releases E2F transcription factor -> free E2F allows progression of cell cycle G1 -> S

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41
Q

germline mutation of Rb results in which 2 cancers?

A

retinoblastoma

osteosarcoma

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42
Q

what does P53 normally do in response to DNA damage (not repairable)?

A

p53 upregulates BAX -> disrupts Bcl2 -> mitochondrial membrane unstable -> cytochrome c leaks out

also induces p21 which inhibits CDKs (which normally phosphorylate Rb) ->

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43
Q

what chromsomes are Bcl2 and Ig heavy chain locus genes on?

A

Bcl2 on ch 18
Ig heavy chain ch 14

so in follicular lymphoma where t(14;18), the Bcl2 (18) gets translocated to Ig heavy chain locus (14) -> overexpressed -> decreased apoptosis -> lymphoma

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44
Q

what are the 4 carcinomas that spread hematogenously? (most carcinomas spread by lymph)

A
  1. renal cell carcioma (renal vein)
  2. HCC (hepatic vein)
  3. follicular carcinoma of thyroid
  4. choriocarcinoma (trophoblasts. it’s their job lol)
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45
Q

granulomas release what enzyme that causes hyper vitD - > hypercalcemia? like in sarcoidosis

A

1-alpha hydroxylase

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46
Q

marker -> tissue type:

vimentin -> ______
desmin -> _____
_____->neuroglia
_____->neurons

chromogranin and ____-> neuroendocrine cells
S-100 -> ____________(3)

A

vimentin -> mesenchyme
desmin -> muscle
GFAP -> neuroglia
neurofilament -> neurons

chromogranin and synaptophysin-> neuroendocrine cells
S-100 -> melanoma, Schwannoma, Langerhans cell histiocytosis

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47
Q

radon accumulates in ______

exposure increases risk of ____ cancer

A

closed spaces, like basements

lung cancer (2nd leading cause after smoking!)

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48
Q

5 cancers that have psammoma bodies (concentric dystrophic calcification)

A 
Papillary carcinoma of thyroid
ƒ Serous papillary cystadenocarcinoma of ovary
ƒ Meningioma
ƒ Malignant Mesothelioma
endometrial carcinoma
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49
Q

common primary sites that metastasize to brain (5)

A

lung > breast > kidney, colon, melanoma

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50
Q

common primary sites that metastasize to bone (lead kettle = Pb KTL)

A

Prostate, Breast > Kidney, Thyroid, Lung.

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51
Q

3 organs where problems can lead to increased alkaline phosphatase

A

liver- obstruction or metastasis
bone - dz or metastasis
Seminoma - placental ALP

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52
Q

4 cancer/tumors that have elevated alpha-fetoprotein

A

HCC
hepatoblastoma
yolk sac (endodermal sinus) tumor
mixed germ cell tumor

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53
Q

4 cancer/tumors that have elevated B-hCG

A

hydatidiform moles
choriocarciomas (getational trophpoblastic dz)
testicular cancer
mixed germ cell tumor

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54
Q

serum marker for pancreatic adenocarcinoma

A

CA 19-9

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55
Q

serum marker for ovarian cancer

A

CA 125

56
Q

CA 15-3/CA 27-29 is serum marker for what?

A

breast cancer

57
Q

P-glycoprotein is marker for what?

A

it’s a multidrug resistance protein. pumps out the drugs out of the cell

58
Q

Cachexia is mediated by what 4 cytokines?

A

TNF
IFN-y
IL-1
IL-6

59
Q

Hb electrophoresis in order fromr least to farthest traveling Hb

A

C S F A

60
Q

genetic mutation of HbS? HbC?

A

HbS = glutamic acid replaced with valine (neutral charge)

HbC: glutamic acid replaced with lysine (+ charge)

61
Q

the 2 principal targets of antithrombin (and thus heparin)

A

thrombin

factor Xa

62
Q

2 sources of vWF

A

Weibel Palade bodies of endothelial cells

alpha-granules of platelets

63
Q

hemostasis: platelets bind to vWF via _____ receptor

A

GpIb

64
Q

what does alpha-granules put out? (3)

A

vWF
fibrinogen
fibronectin

65
Q

what do you give to prevent mast cell degranulation. used for asthma prophylaxis

A

cromolyn sodium

66
Q

ADP binds to ____ receptor to induce GPIIb/IIIa expression on platelets

A

P2Y12

67
Q

what is Ristocetin test? postive test in what? (2)

A

Ristocetin activates vWF to bind GpIb on platelets.

failure (positive test) occurs in von Willebrand dz and Bernard Soulier syndrome (missing Gp1b receptor)

68
Q

when do you see spur cells

A

liver dz

abetalipoproteinemia, or any other state of cholesterol dysregulation (increased cholesterol in RBCs)

69
Q

when do you see echinocytes (burr cells)? (3)

A

end stage renal dz
liver dz
pyruvate kinase deficiency

70
Q

ringed sideroblasts is from excess ____ in ___(organelle)

A

iron in mitochondria

71
Q

when do you see target cells (HALT the hunter said to his target)

A 
caused from decreased cytoplasm volume of increased membrane so
 HbC dz, 
Asplenia, 
Liver dz, 
Thalassemia
72
Q

how do Heinz bodies form?

A

oxidation of Hb-SH groups to -S–S -> Hb precipitates as Heinz bodies

73
Q

___ and ___ are released from platelet dense granules

A

Ca2+ and ADP

74
Q 
mucosal bleeding:
epistaxis
hemoptysis
GI bleeding
hematuria
menorrhagia
intracranial bleeding

is this primary or 2ndry hemostasis disorder?

A

primary

also have skin bleeding: purpura, bruising, ecchymoses

75
Q

Immune thrombocytopenic purpura, pt has Ig__ against platelet antigens, produced by_______

A

IgG (which means it can cross placenta if woman is pregnant)

plasma cells in spleen

76
Q

decreased ADAMTS13 enzyme results in what dz? what is its normal function?

A

thrombotic thrombocytopenic purpura (TTP)

enzyme fx is to cleave vWF into monomers for degredgation

77
Q

E. coli O157:H7 releases _____ which damages endothelial cells resulting in platelet microthrombi

A

verotoxin

78
Q

mild thrombocytopenia an enlarged platelets, abnormal Ristocetin test. dx and pathogenesis?

A

Bernard Soulier sydrome
GP1b deficiency
there is thrombocytopenia bc the platelets die faster

79
Q

extrinsic coagulation pathway is activated by? measure by (PT or PTT)?

A

activated by tissue thromboplastin (activates factor VII)

measure by PT

80
Q

obstetric complications can cause DIC b/c of what enzyme in the amniotic fluid?

A

tissue thromboplastin

81
Q

if pt has hemophilia like symptoms (deep bleeding) and mixing study normalizes PTT, pt has ________
if mixing study does not correct patient’s PTT, pt has __________

A

PTT normalizes: hemophilia. deficiency.

does not normalize: coagulation factor inhibitor. most commonly anti-FVIII antibody.

82
Q

tx for von willebrand dz is ____, which increases vWF release from _______

A

desmopressin (ADH analog)

from Weibel-Palade bodies

83
Q

what events can cause DIC, besides the obvious? (trauma, obstetric, gram neg sepsis)

A 
Pancreatitis, 
Malignancy (Adenocarcioma the mucin activates coagulation, and APL the primary granules activate coagulation), 
Nephrotic syndrome, 
Transfusion 
Rattlesnake bite
84
Q

radical prostatectomy releases urokinase which activates ______, leading to ______

A

urokinase activates plasmin -> to much fibrinolysis. bleeding.

85
Q

how do labs differ for DIC vs fibrinolysis disorder/dysfunction (like from overactive plasmin)?

A

fibrinolysis dysfunction has normal platelet count and does not have elevated D-dimer

DIC has decreased platelet count and elevated D-dimer

both have increased PT PTT and bleeding time

86
Q

liver cirrhosis causes fibrinolysis disorder how?

A

reduced production of alpha2-antiplasmin -> overactive plasmin

87
Q

what does aminocaproic acid do?

A

blocks activation of plasminogen. give to patients with fibrinolysis dysfunction

88
Q

what do endothelial cells produce/secrete to prevent thrombosis? (5)

A 
PGI2
NO
heparin like molecules -> augment ATIII
tPA
thrombomodulin - redirects thrombin to activate protein C -> protein C inactivates factors V and VIII
89
Q

person has vessel thrombosis, mental retardation, lens dislocation, and long slender fingers. they have high homocysteine levels. what enzyme is deficient?

A

cystathionin . beta synthase (CBS) deficiency. normally turns homocysteine to cystathionine. deficiency leads to homocysteine buildup and the following sx

90
Q

what conditions have high levels of D-dimer? (3)

A

DIC
DVT
PE

91
Q

which section of small bowel is iron, folate, and B12 absorbed in?

A

iron - duodenum
folate B9 - jejunum
B12 - ileum

it’s in order of increasing #. (think of Fe as 0. or even 2-3)

92
Q

which enzyme is deficient in porphyria cutanea tarda? presentation?

A

uroporphyrinogen decarboxlyase

neuro stuff. photosensitivity -> skin blistering
exacerbated by alcohol

93
Q

which enzyme is deficient in acute intermittent porphyria? presentation? (the 5 P’s)

A

PBG deaminase

sx are the 5 P’s: Painful abdomen, Port wine-colored urine, Polyneuropathy, Psychological disturbances, Precipitated by drugs, alcohol, starvation

94
Q

tx for acute intermittent porphyria?

A

glucose and heme, bc they inhibit ALA synthase

95
Q

basophilic stippling in cells is from _______

A

residual ribosomes

96
Q

how does preganancy and oral contraceptives affect TIBC and % saturation iron in blood?

A

increase TIBC

decrease % saturation

97
Q

4 common protein deficiencies in hereditary spherocytosis

A

akyrin
spectrin
band 3.1
protein 4.2

98
Q

what blood thing has increased mean corpuscular Hb concentration (MCHC)?

confirm dx with?

A

hereditary spherocytosis. b/c cells are smaller bc less membrane, but normal amt of cytoplasm -> increased Hb concentration relatively

confirm dx with osmotic fragility test

99
Q

treat sickle cell anemia with ____ b/c it increases levels of HbF

A

Hydroxyurea

100
Q

which cancer is a complication of PNH?

A

AML. makes sense, they’re both RBC stuff

101
Q

mix and match:

IgM or IgG causes intravascular or extravascular hemolysis?

A

IgG = extravascular hemolysis

IgM = intravascular hemolysis

102
Q

3 common causes of immune hemolytic anemia extravascular hemolysis

A

SLE
CLL
drugs - penicillin and cephalosporins

103
Q

which plasmodium is every day fever?

A

P. falciparum

104
Q

infectious mononucleosis is not actually reacting monocytes, it’s _____ cells

virus can become dormant in _____ cells

A

reacting CD8+ T cells

virus can become dormant in B cells -> recurrence and/or B cell lymphoma

105
Q

lymphoblasts have positive nuclear staining for ____ a DNA polymerase

A

TdT

106
Q

if cells are TdT positive, you know it’s ____ leukemia

A

ALL

TdT is only present in lymphoblasts

107
Q

which leukemia arises in children before and after 5 yrs old

A

> 5 yrs old = ALL

< 5 yrs old = AML, specifically acute megakaryoblastic leukemia

108
Q

which 2 places does ALL like to metastasize to?

A

scrotum

CNS (meninges, CSF)

109
Q

which translocations have poor and good prognoses for ALL?

A

good prognosis: t(12;21)

bad prognosis: t(9;22) Philadelphia + BCR-ABL fusion. remember this is seen a lot in CML! in which case it’s doesn’t mean bad prognosis

110
Q

which cancer usually presents in teenagers as a mediastinal (thymic) mass?

A

T-cell ALL (forms mass so called lymphoma even tho ALL is a leukemia)

the 3 T’s. T-cell, Teenager, Thymic mass

mediastinal mass can result in SVC like syndrome

111
Q

if you see Auer rods in cancer cells, those are made of _____ and means it’s a ____ leukemia

A

myeloperoxidase

AML (not seen in CML i guess). more specifically APL type.

112
Q

APL of AML has what translocation?

A

t(15;17) = retinoic acid RAR disruption. all trans retinoic acid (ATRA) is effective tx

113
Q

t(1;22) is for what leukemia? who is at risk of getting this?

A

acute megakaryoblastic leukemia of AML.

Down’s kids . <5 yrs old

114
Q

AML risk increases with prior exposure to what 3 substances/things

A

alkylating agents
radiotherapy
benzene

115
Q

smudge cells. which leukemia? which 2 CDs coexpressed?

A

CLL
CD5 and CD20 ( can have others also, it’s B cell)

CD5 is normally on T cells! so this is unique.

116
Q

guy has leukemia. he also has hypogammaglobulinemia and autoimmune hemolytic anemia. which leukemia does he have?

A

CLL. coexpresses CD5 and CD20, btw

117
Q

adult T cell leuk/lymph is proliferation of which T cells?

presentation?

A

mature CD4+ T cells

skin lesions, lytic bone lesions w/ hypercalcemia (kinda looks like MM) + lymphadenopathy, hepatosplenomegaly

118
Q

blood smear shows lymphocytes with cerebriform nuclei (nuclei has brain like looking folds/contour)

which cancer? which cells are proliferation?

A

mycosis fungoides disseminated into Sezary syndrome.

CD4+ T cells

119
Q

what is unique about the splenomegaly that happens in hairy cell leukemia? what other unique manifestation do you get?

A

due to accumulation of hairy cells in red pulp. b/c usually leukemias are in white pulp.

also dry tap on bone marrow aspiration due to marrow fibrosis.

AND lymphadenopathy is usually absent!

120
Q

differences between CML and acute leukemoid reaction?

A

CML is negative for leukocyte alkaline phsophatase (LAP) stain, and CML has increased basophils. and the translocation, obvis

leukemoid rxn is opposite of those

121
Q

what is the mediator of marrow fibrosis in myelofibrosis?

A

excess PDGF derived from megakaryocytes

122
Q

gene translocation in follicular lymphoma?

A

t(14;18)

Bcl2 on 18 translocates to Ig heavy chain locus on 14

123
Q

important difference histologically between folicular lymphoma and follicular hyperplasia

A

follicular hyperplasia has tingible body macrophages present in germinal centers

124
Q

gene translocation in mantle cell lymphoma?

A

t(11;14)

cyclin D1 on 11 translocates to Ig heavy chain locus on 14 (remember cyclin D1 poromotes G1/S transition)

125
Q

gene translocation in Burkitt’s lymphoma?

A

t(8;14)

c-myc on 8 to heavy chain 14

126
Q

which interleukin can be high in multiple myeloma?

A

IL-6. it stimulates plasma cell growth and Ig production

127
Q

what are the lymphoma cells in waldenstrom macroglobulinemia?

A

B cell lymphoma with monoclonal IgM production (IgM is big hence macroglobulinemia in name)

128
Q

M spike in waldenstrom macroglobulinemia is from Ig____ whereas M spike in multiple myeloma is Ig___ and Ig___

A

WM: IgM
MM: IgG and IgA

129
Q

Langerhans cells have Birbeck granules (tennis racket) and are ___ and ____ positive by immunohistochemistry.

derived from what cell?

A

CD1a and S100 positive

derived from bone marrow monocytes, they are dendritic cells mostly in skin

130
Q

classic presentation of Hand-Schuller-Christian dz:

remember, it has names in it so it’s malignant. and malignant ones have rash. also it has 3 names so affects children > 3 yrs old

A 
scalp rash
lytic skull
diabetes insipidus
exophthalmos
recurrent otitis media
131
Q

child presents w/ failure to thrive, developmental delay, and megaloblastic anemia. treat them w/ folate and B12 but doesn’t get better. what is it? tx?

A

orotic aciduria. defect in UMP synthase (orotic acid -> UMP in de novo pyrimidine synthesis pathway)

will have orotic acid in urine, but NO hyperammonemia, as opposed to OTC deficiency that has increased orotic acid and hyperammonemia

tx: give uridine triacetate to bypass mutated enzyme

132
Q

<1 yr old baby has macrocytic anemia (not megaloblastic). craniofacial abnormalities, TRIPHALANGEAL THUMBS and other upper extremity malformations. what do they have? pathogenesis?

A

Diamond Blackfan anemia

intrinsic defect in erythroid progenitor cells

increased %HbF but decreased total Hb

133
Q

kid has normocytic anemia. short stature. cafe au lait spots, hypoplastic thumbs. what do they have? pathogenesis?

A

Fanconi anemia

due to DNA repair defect -> bone marrow failure. can see macrocytosis though. so it’s similar to diamond blackfan anemia in presentation. this is a type of aplastic anemia.

134
Q

how does pyruvate kinase deficiency cause hemolytic anemia? presentation?

A

rxn is PEP -> pyruvate

dec enzyme -> decreased ATP -> rigid RBCs -> extravascular hemolysis
(also, increases 2,3-BPG levels -> right shift)

hemolytic anemia in a newborn

135
Q

which cancers is RET oncogene mutation associated w/? (2)

A

medullary thyroid cancer

pheo

STEP1 (10 decks)

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