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Step 1 Andre > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

t(9;22)

A

philadelphia chromosome

chronic myelogenous leukemia (CML)

BCR-ABL fusion protein

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2
Q

Burkitt Lymphoma

A

t(8;14) t(8;22) t(8;2)

movement of myc protooncogene from chromsome 8 to regions near Ig promoter sites on chromosomes 14, 22, or 2

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3
Q

t(14;18)

A

non-Hodgkin folliculate small cleaved cell lymphoma

bcl-2 protooncogene is sent from Chr 18 to Ig heavy chain promoted on chr 14

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4
Q

inv(16)

A

M4Eo (eosinophilic) subtype of AML

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5
Q

t(15;17)

A

acute promyelocytic leukemia

hemorrhage 2/2 intravascular coagulation and increased fibrinolysis

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6
Q

bcr-abl

A

t(9;22)

philadelphia chromosome

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7
Q

myc

A

on chromosome 8

t(8;22) t(8;2) t(8;14)

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8
Q

bcl-2

A

t(14;18)

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9
Q

Germinoma

  • most common location or brain germinoma?
  • classic sxs
A
  • pineal region
  • precocious puberty, Parinaud syndrome, obtructive hydrocephalus

(Parinaud = paralysis of upward gaze - dorsal midbrain syndrome)

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10
Q

pilocytic astrocytoma

  • microscopically
  • cells are mixed with?
  • affects where
A
  • spindle-cells w/hair-like glial processes a/w microcysts
  • Rosenthal fibers and granular eosiniophilic bodies
  • cerebellum, brainstem, hypothalamus, optic
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11
Q

rate limiting enzyme of heme synthesis

A

delta-ALA synthase (glycine and succinyl-coa into delta-ALA)

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12
Q

lead affects what enzymes

  • what accumulates
  • sxs
A

Ferrochelatase
ALA dehydratase

  • protoporphyrin and delta-ALA
  • microcytic anemia, MR, h/a, demyelination
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13
Q

Acute intermittent porphyria

  • what enzymes affected
  • what accumulates
  • sxs
  • tx
  • why does that work
A
  • porphobilinogen (PBG) deaminase
  • porphobillinogen (PBG), delta-ALA
  • 5 P’s: Painful abd, Port wine urine, Polyneuropathy, Psycho distrubances, Precipitated by drugs etc
  • glucose and heme
  • inhibits ALA synthase
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14
Q

Porphyria cutanea tarda

  • what enzyme affected
  • what accumulates
A
  • uroporphyrinogen decarboxylase

- uroporphyrin

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15
Q

what’s most common porphyria

A

porphyria cutanea tarda

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16
Q

heme and ALA synthase relationship

A

decrease heme –> increase ALA synthase

increase heme –> decrease ALA synthase

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17
Q

squamous cell carcinoma pathology (3)

A
  1. polygonal cells
  2. eosinophilic cytoplasm
  3. keratin-pearls
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18
Q

bronchioloalveolar carcinoma pathology (3)

effect on underlying lung archicutre

A
  1. tall, columnar cells
  2. spread along alveolar septae
  3. papilalry projections into alveolar spaces

underlying architecutre typically preserved

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19
Q

papillary thyroid carcinoma pathology (3)

A
  1. branching papillae
  2. fibrovascular stalk covered by neoplastic cuboidal cells
  3. ground glass nuclei
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20
Q

small cell lung carcinoma pathology (4)

A
  1. undifferentiated oat cell
  2. centrally located
  3. from primitive cells of basal layer of bronchial epithelium
  4. round or oval cells w/scant cytoplasm and large hyperchromatic nuclei
    - resemble lymphocytes but smaller
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21
Q

BCR-ABL in what d/o

A

CML

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22
Q

tx for CML

A

gleevec = Imatinib

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23
Q

c-myc is what d/o

A

Burkitt lymphoma

24
Q

APC

  • what chromosome
  • what function
  • role in cancer
A
  • chromosome 5
  • cell growth and adhesion
  • APC mutation is first step in adenoma-carcinoma sequence
25
Q

APC found in what sorts of cancers

A

most sporadic colon cancer

all familial polyposis syndromes

26
Q

DCC = ?

A

deleted in colon cancer

27
Q

DCC role in cancer

A

final step of progression of large adenomatous polyps into adenocarcinoma

28
Q

k-ras in colon cancer

A

it’s an onco-gene that is responsible for size increases in adenomatous polyps

29
Q

p53 in colon cancer

A

triggers final step of the adenoma-to-carcinoma sequence

30
Q

MSH-2

  • what is it
  • involvement in cancer
A

DNA mismatch repair gene

HNPCC or Lynch syndrome

31
Q

M-0 AML

  • differentiation
  • description
A

minimally differentiated

very immature, few or no Auer rods

32
Q

M1 AML

  1. differentiation
  2. description
A
  1. AML w/out differentiation

2. very immature, few or no Auer rods

33
Q

M2 AML

  1. differnetiation
  2. description
A
  1. AML w/maturation
    • auer rods; + peroxidase

t(8;21)

34
Q

M3 AML

  1. differentiation
  2. description
A
  1. Acute promyelocytic leuekmia
  2. lots of Auer rods; + peroxidase

t(15;17)

young pts and DIC is common

35
Q

M4 AML

  1. differentiation
  2. description

PX?

A
  1. Acute myelocytic leukemia
  2. monoblasts and promonocytes
    - negative peroxidase
    + nonspecitic esterase

poor Px

36
Q

M5 AML

  1. differentiation
  2. description
A
  1. acute monocytic leukemia
  2. monoblasts and promonocytes
    - negative peroxidase
    + nonspecific esterase
37
Q

M6 AML

  1. differentiation
  2. description
A
  1. Acute erythroleukemia

2. + PAS staining erythroblasts

38
Q

M7 AML

  1. differentiation
  2. description
A
  1. acute megakaryocytic leukemia
    • platelet peroxidase
      rare and poor Px
39
Q

Mantle cell lymphoma

  • mutation
  • what cell
A

t(11;14)

B cell malignancy

40
Q

t(11;14)

  • what malignancy
  • what does it code for
A
  • Mantle cell lymphoma

- cyclin D activation

41
Q

del 13q sen in what

A

CLL

42
Q

HbS mutation

A

2 normal alpha chains
2 mutated beta chains
D for V at 6 position

43
Q

HbC mutation

A

D for K –> mild chronic hemolytic anemia

44
Q

what is JAK2

A

tyr kinase mediated receptor – STAT

45
Q

disorders with JAK2 mutation (3)

A
  1. essential thrombocytosis
  2. polycythemia vera
  3. primary myelofibrosis
46
Q

diagnostic features of polycythemia vera (6)

A
  1. pruritus
  2. erythromelalgia
  3. splenomegaly
  4. thrombotic complications
  5. erythrocytosis
  6. trhombocytosis
47
Q

diagnostic features of primary myelofibrosis (5)

A
  1. severe fatiuge
  2. splenomegaly to the point of causing early satiety
  3. hepatomegaly
  4. anemia
  5. BM fibrosis
48
Q

CML leukocytosis what shift

A

left shift (more myelocytes, metamyelocyte,s band forms)

49
Q

IL-1

  • what is it
  • what malignancy secretes it
A

osteoclast activating factor

multiple myeloma

50
Q

IL-6

  • what is it
  • what malignancy secretes it
A

a factor secreted by multiple myeloma cells that leads to bone resoprtion

51
Q

what causes the fatigue, confusion, and constipation in MM pts

A

hypercalcemia from bone resroption

52
Q

hypercoagulability seen commonly in what cancers

A

adenoCA of pancreas, colon, lung

53
Q

pathogenesis of hypercoagulability neoplastic syndrome

A

adnoeCA prodcues thromboplastin-like substance that can cause chronic intravascular coagulation

54
Q

what is Trousseau’s syndrome?

A

migratory superficial thrombophlebitis

55
Q

what should you be wary about if you see Trousseau’s syndrome?

A

visceral cancer

56
Q

properties of anaplastic tumors (5)

A
  1. loss of cell polarity, so disorganized in infiltrative fashion
  2. variation in shape and size of cells (pleomorphism) and nuclei
  3. disproportionately large nuclei w/abundant coarse chromatin and large nucleoli
  4. numerous, often abnormal mitotic figures
  5. giant multnucleated tumor cells

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