AAW - Heme/Onc

Question 1

cancers assc with EBV

Answer 1

Associated with Hodgkin lymphoma, endemic Burkitt lymphoma (seen most commonly in kids and involves the jaw), nasopharyngeal carcinoma.

Question 2

burkitt lymphoma general

Answer 2

c-myc gain of function (oncogene)

t(8;14)—translocation
of c-myc (8) and
heavy-chain Ig (14)

Starry sky appearance - sheets of lymphocytes with interspersed macrophages

jaw lesions in endemic form in Africa,; pelvis or abdomen in sporadic form

Question 3

Diffuse large B-cell lymphoma general

Answer 3

usually in older adults

t(14;18)

most common type of non-Hodgkin lymphoma in adults

Question 4

Mantle cell lymphoma general

Answer 4

I am an older male with 5 CDs on my Mantle which makes it Super LLegit/CooLL so don’t Smudge them (my mantle is above the TV where I am watching the movie “11:14” (google it))

Mantle cell lymphoma and Small Lymphocytic Lymphoma/Chronic Lymphoid Leukemia (with smudge cells) are CD5+

t(11;14)

Question 5

follicular lymphoma general

Answer 5

adults

t(14;18) translocation of heavy chain Ig and BCL-2

BCL-2 inhibits apoptosis

Question 6

all the translocations of non-hodgkin lympoma

Answer 6

burkitt, mantle, diffuse/follicular

8;14
11;14
14;18

kind of a pattern

Question 7

Adult T-cell lymphoma general

Answer 7

caused by HTLV-1 (assc with IV drug abuse)

adults present with cutaneous lesions, lymphadenopathy, hepatosplenomegaly, lytic bone lesions, hypercalcemia

endemic in japan, west africa, caribbean

Question 8

mycosis fungoides/sezary syndrome general

Answer 8

adults- cutaneous t-cell lymphoma

present with cutaneous crusty red patches

circulating malignant cells seen in sezary syndrome

indolent, CD4+

Question 9

virus assc with cervical, anal, penile carcinoma

Answer 9

HPV
HPV 6 and 11 are low risk
consider everything else high risk for the high yield only

the warts that HPV causes are called verrucae: Soft, tan-colored, cauliflower-like papules

genital warts = condyloma acuminatum

Question 10

hepatocellular carcinoma assc virus

Answer 10

chronic hep B/C virus

Question 11

kaposi sarcoma assc virus

Answer 11

HHV8

Question 12

burkitt lymphoma oncogene

Answer 12

c-myc

gene product is transcription factor

also t(8:14)

(mantle t(11;14), diffuse and follicular lymphoma t(14;18))

Question 13

patient goes into starvation mode and they get:

painful abdomen, hallucinations, dark red colored urine

Answer 13

Acute intermittent prophyria
deficiency in porphybilinogen deaminase
accumulated prophobilinogen in the urine

Symptoms (5 P’s):
ƒ Painful abdomen
ƒ Port wine–colored urine
ƒ Polyneuropathy
ƒ Psychological disturbances
ƒ Precipitated by drugs, alcohol, and starvation

Question 14

very young patient presents with large mass in the inferior pole of the retina

what cancer
what other cancers go with it
what gene

Answer 14

retinoblastoma

osteosarcoma

Rb gene deletion (gene product inhibits E2F, blocking G1 ->S phase)

Question 15

bleomycin

Answer 15

intercalates DNA during G2, causes scission by an oxidative process

pulmonary side-effects

Question 16

cyclophosphamide

Answer 16

DNA alkylator - crosslinks DNA at any stage of cell cycle

activated by cytochrome P450

Question 17

vincristine is mainly used for

Answer 17

lymphomas

Question 18

most common metastatic tumors to the peritoneum

Answer 18

ovarian and pancreatic carcinoma

Question 19

african american with who gets jaundice, fatigue, SOB after taking a drug

what is most likely enzyme deficiency

what are some possible drugs

Answer 19

g6pd

drugs that cause ROS (no G6PD means no HMP shunt which means no NADPH for glutathione which means oxygen radicals in RBCs reign supreme)

Question 20

welder presents with microcytic anemia and basophillic stippling

what is affected

Answer 20

lead poisoning

Ferrochetalase (binds iron to heme) and delta-aminolevulinic acid dehydratase enzymes (ALA dehydratase)

accumulated protoporphyrin, delta-ALA in the blood

Can mimic a porphyria

Question 21

factors involved in the extrinsic pathway

Answer 21

I, II, V, VII, X

Question 22

hemophilia a,b,c

Answer 22

all have increased PTT and normal PT (intrinsic pathway disorder)

ABC
8,9,11

a: deficiency of factor VIII; x-linked recessive
b: deficiency of factor IX; x-linked recessive
c: deficiency of factor XI; autosomal recessive in ashkenazi jews

Sx: macrohemorrhage, hemarthroses (bleeding into joints), easy bruising.

Question 23

retinoblastoma in a kid

what other cancer are they at risk for

Answer 23

osteosarcoma

both controlled by the RB gene

Question 24

osteochondroma

Answer 24

most common benign bone tumor

males less that 25 years old

bony exostosis with cartilaginous cap

rarely transforms into chondrosarcoma

Question 25

giant cell tumor

Answer 25

benign but locally aggressive

20-40 years old

epiphyseal end of long bones, often around knee

aka “osteoclastoma”

soap bubble on x-ray

Question 26

osteosarcoma

Answer 26

2nd most common primary malignant bone tumor after MM

bimodal distribution, 10-20 years old, greater that 65

Predisposing factors: Paget disease of bone, bone
infarcts, radiation, familial retinoblastoma,
Li-Fraumeni syndrome (germline p53
mutation

metaphysis (between epiphysis and diaphysis) of long bones, often around knee

elevation of periosteum or sunburst pattern on x-ray

Question 27

what chemotherapy’s side effects are made better by leucovorin?

which drug’s side effects are made worse

Answer 27

with MTX, myelosuppression is reversible with leucovorin “rescue”

with 5-FU, myelosuppression is worsened with leucovorin

leucovorin = folinic acid

Question 28

nighttime bone pain, raised lesion in the diaphysis of a long bone

Answer 28

osteoid osteoma

Question 29

“pseudopalisading” pleomorphic tumor cells that border central areas of necrosis and hemorrhage

what cancer
what cells stain for GFAP

Answer 29

glioblastoma multiforme

(grade IV astrocytoma)

astrocytes stain for GFAP

Question 30

brain tumor of arachnoid cells

spindle cells concentrically arranged in a whorled pattern

Answer 30

Meningioma - spindle cells concentrically arranged in a whorled pattern is psammoma bodies

its external to brain parenchyma, may have dural attachment

Question 31

cerebellar

closely arranged, thin-walled capillaries with minimal intervening parenchyma

what tumor
paraneoplastic effects?
assc with what disease?

Answer 31

hemangioblastoma

can produce erythropoietin leading to polycythemia

VHL syndrome with found with retinal angiomas

Question 32

cancers that are S-100 positive

Answer 32

mesodermal origin cancers

langerhans cell histiocytosis

Melanoma

Schwannoma (classically at the cerebellopontine angle, but can be in any peripheral nerve. bilateral vestibular schwannomas found in NF-2)

Question 33

cerebellopontine angle tumor

Answer 33

schwannoma, classically

S-100 +

Question 34

frontal lobe calcification

lots of thin capillaries, round nuclei with clear cytoplasm,

Answer 34

oligodendroglioma

rare, slow growing

“fried egg cells”

Question 35

trousseau sign

Answer 35

migratory thrombophlebitis - redness and tenderness on palpation of extremities

seen in pancreatic carcinoma (producing tissue factor)

Question 36

treatment regimen for hodgkin’s lymphoma

Answer 36

DVBD

doxorubicin
vinblastine
bleomycin
dacarbazine

Question 37

3 year old
abdominal mass
big tounge
big organs
one side of the body is bigger than the other

what chromosome is affected

what is changed on that chromosome

Answer 37

beckwith-wiedemann: wilms tumor, macroglossia, organomegaly, hemihypertrophy

deletion of the WT2 tumor suppressor gene on chromosome 11 (just wilms tumor is deletion of WT1 on chromosome 11)

Question 38

recombinant IL-2 is used to treat what

Answer 38

RCC, metastatic melanoma