STEP 2 Heme/Onc Flashcards

1
Q

NF-1 features (inheritance, chromosome, clinical)

A

AD - von Recklinghausen dz
Chromosome 17 neurofibromin
cafe-au-lait spots, iris hamartomas (Lisch nodules), neurofibromas (peripheral nerve sheath tumors - optic pathway), +brain tumor incidence

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2
Q

NF-2 features (inheritance, chromosome, clinical)

A

AD - central neurofibromatosis - MISME
Chromosome 22 merlin
Bilateral acoustic schwannomas, meningiomas, schwannomas

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3
Q

TS complex (inheritance, chromosome, clinical)

A

AD - TSC1/TSC2
hamartin/tuberin chr 9/16
Cortical {tubers, hamartomas, subependymal giant cell astrocytomas}, ash leaf spots, angiofibromas, renal angioleiomyomas, MR, seizures

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4
Q

Non-beta-cell tumors?

A

Large mesenchymal tumors

Produce IGF-II (no c-peptide or insulin)

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5
Q

Transfusion reactions: nonhemolytic - mechanism, onset, sx, tx

A

nonhemolytic febrile (most common): cytokine formation during storage
- 1-6hrs
- fevers, chills, etc
- stop transfusion give tylenol
TRALI: donor anti-leukocyte abx (usu. from preg.)
- 0-6hrs
- noncardiogenic pulmonary edema, bilateral infiltrates on CXR
- stop transfusion, resp support
allergic: reaction to donor proteins
- IgA deficient recipient -> +anaphylaxis risk
- seconds to minutes
- urticaria (minor), anaphylaxis (airway edema, hypotn - severe)
- antihistamines. if severe, stop transfusion, give IM epi, glucocorticoids, antihistamines, use washed RBCs and IgA free plasma next time
primary hypotensive: donor bradykinin
- incr risk if on ACEI
- within minutes

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6
Q

von Hippel Lindau features?

A
phakomatosis
FAMILY HISTORY (AD)
hemangioblastomas
RCC
pheos
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7
Q

Sturge-Weber features?

A

phakomatosis - somatic mutation in GNAQ gene
NOT HERITABLE
port wine stain over trigeminal nerve territory
MR
seizures
visual impairment

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8
Q

McCune-Albright features?

A
mosaic in cAMP-kinase gene
3 P's:
polyostotic fibrous dysplasia
precocious puberty
pigmentation (irregular border cafe au lait)
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9
Q

HIT type 1 vs type 2

A
type I: drop in plt via direct action of heparin on plt. Normalizes w/in 2 days.
type II: anti-PF4 complex abx vs plts
plt drop > 50% nadir 30-60
5-10days
arterial and venous thrombosis
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10
Q

thal minor vs iron deficiency anemia

A

thal minor: nl RDW, nl RBCs, target cells

fe deficiency anemia: high RDW, low RBCs

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11
Q

Smear findings: howell-jolly bodies?

A

Remnant of RBC nucleus.

Seen in cases of functional asplenia (eg sicklers) or splenectomy

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12
Q

Helmet cells?

A

microangiopathy (DIC, HUS, TTP)

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13
Q

Basophilic stippling?

A
Ribosomal precipitates (blue granules in cytoplasm)
seen in thalassemias and heavy metal poisoning
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14
Q

Heinz bodies?

A

aggregates of denatured Hgb
seen in G6PD and thalassemia
accompanied by bite cells (when macrophages bite them off)

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15
Q

Transfusion reaction: hemolytic

A

acute hemolytic: ABO incompatibility, 1-hr timescale
-fever, chills, flank pain, renal failure, DIC
-pink plasma, +Coombs
delayed hemolytic: anamnestic response 2-10d
- +direct coombs

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16
Q

Thyroid cancers (4)

A

medullary: secretes calcitonin, MEN2A/B
papillary: orphan annie-eyes, psammoma bodies, unencapsulated, good prognosis, most common
follicular: early hematogenous spread, encapsulated, carcinoma invades
anaplastic: you’re screwed

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17
Q

trastuzumab tox?

A

cardiac, get an echo

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18
Q

classic triad for brain abscess

risk factors for brain abscess

A

fever, nocturnal or morning headaches, focal deficit, +- seizure
sinus, dental infx, otitis media/mastoiditis
cyanotic congenital heart dz (R->L shunt)

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19
Q

glucagonoma: what does NEC look like?

is there DM?

A

NEC - erythematous plaques with eroded borders and central clearing
DM is mild

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20
Q

mediastinal masses - anterior, middle, posterior

A

anterior: 5T’s
- thyroid (retrosternal)
- thymus
- thoracic aorta
- terrible lymphoma
- teratoma
middle:
- bronchogenic cyst
posterior:
- esophageal leiomyoma
- neuroblastoma

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21
Q

Waldenstroms vs MM ddx

A
Waldenstroms: hyperviscosity IgM
- splenomegaly
- retinal vein engorgement -> visual sx
- headache and dizziness
- pain and numbness in extremities
MM: usually IgA or IgG
- hypercalcemia
- renal failure
- anemia
- bence-jones proteinuria
- lytic bone lesions
- infections
22
Q

lung cancers and paraneoplastic syndromes: SCLC, squamous, adeno

A

SCLC: SIADH, ectopic 2ary Cushing’s (pigmentation!), Lambert-Eaton

squamous: hypercalcemia, cavitary lesion
adeno: (usu peripherally located lesion in lung): hypertrophic osteoarthropathy

23
Q

cauda equina syndrome vs conus medullaris syndrome?

A

cauda equina - spinal nerve roots
- radicular distribution of pain (unilateral)
- dermatomal numbness (unilateral)
- lower motor neuron signs (hyporeflexia)
- saddle anesthesia
- late bowel bladder
conus medullaris - spinal cord
- back pain
- numbness perianal only
- upper and lower motor neuron signs (upgoing toes)
- early bowel bladder

24
Q

hydroxychloroquine side effect?

A

retinopathy

25
Q

AML + halo sign on chest CT?

A

aspergilloma

26
Q

bilateral breast discharge green-brown?

A

galactorrhea

get TSH and prolactin

27
Q

brain mets from lung ca mgmt?

A

NSCLC single met: surgery or stereotactic rads +- WBR
can’t operate: stereotactic rads
multiple mets or poor perf status: WBR
chemosensitive tumor (SCLC, choriocarcinoma, seminoma, lymphoma)

28
Q

complications from sickle cell trait?

A
  • painless hematuria

- hypo or isosthenuria = nocturia, polyuria

29
Q

Esophageal manometry readings for scleroderma?

A

loss of distal peristalsis

30
Q

Which organisms are hemochromatosis pts vulnerable to?

A

Vibrio vulnificus, Yersinia enterocolitica, Listeria

31
Q

CML triad?

A

leukocytosis, basophilia, splenomegaly, BCR-ABL
decreased leukocyte alkaline phosphatase
no lymphadenopathy

32
Q

lung ca hypercalcemia mechanism? Which type of ca?

A

PTHrP - squamous cell ca

33
Q

risk factors for pancreatic ca?

A

first-degree relative with panc ca
BRCA1, BRCA2
Peutz-Jeghers
smoking

34
Q

MGUS vs MM features?

A

serum monoclonal protein 3g cutoff
10% plasma cells in BM cutoff
presence of hypercalcemia, renal dysfunction, anemia, lytic lesions
-elevated beta-2 microglobulin in MM

35
Q

causes of eosinophilia?

A
NAACP:
neoplasm
addison's (adrenal insufficiency)
allergy
collagen vascular dz / cholesterol emboli
parasites
36
Q

indications for low-dose chest CT screening?

A

age 55-80
no medical condition limiting life or willingness to surgerize
current smoker or quit = 30 PY smoking hx

37
Q

causes of type IV RTA (hypoaldosteronism)?

A

diabetes, medications, inherited d/o

  • calcineurin inhibitors
  • NSAIDs
  • ACEI/ARB
  • heparin
38
Q

ITP - history, smear/lab findings, tx

A
  • antecedent viral infection
  • smear: megakaryocytes
  • isolated thrombocytopenia
  • steroids and IVIG if platelets
39
Q

workup of suspected acromegaly?

A

IGF-1 level (should be persistently elevated)

confirm with GH after glucose load

40
Q

HNPCC II extracolonic cancer?

A

endometrial

41
Q

tx for smoke inhalation

A

secure airway - airway edema
CO and HCN poisoning
tx: CO - oxygen (hyperbaric if HbCO > 40%)
HCN - hydroxocobalamin or sodium thiosulfate
-2nd line: induce methemoglobinemia: nitrites

42
Q

indications for specialized RBC tx?

A

irradiated: immunodeficient, blood donated by 1st or 2nd degree relatives
leukoreduced:
-chronically transfused pts
-CMV seronegative with AIDS, transplant
-potential transplant recipients
-previous febrile nonhemolytic transfusion rx
washed:
-IgA deficient
-continued urticarial rxns despite antihistamine
-complement dependent AIHA

43
Q

asbestos exposure + smoking = which cancer?

A

lung (aka “bronchogenic”) carcinoma

pleural mesothelioma less common

44
Q

breast ca: expression of which gene influences tx?

A

HER2: can add trastuzumab

45
Q

staging system for CLL?

A
stage 0 - smudge cells only
stage I - smudge cells + lymphadenopathy
stage II - splenomegaly
stage III - anemia
stage IV - thrombocytopenia - poor prognosis
46
Q

complications of mono?

A

cold AIHA and thrombocytopenia due to serum cold agglutinins
splenic rupture
airway compression d/t tonsillar enlargement

47
Q

can G6PD activity be normal in a G6PD patient?
inheritance of G6PD?
which cells?

A

yes - during an acute attack
X-linked
bite cells

48
Q

osteoporosis risk factors?

A
advanced age
thin body
smoking
alcohol
steroids
menopause
malnutrition
family history
asian or caucasian ethnicity
49
Q

in which 2 conditions can you see spherocytes?

A

AIHA - negative FHx, positive coombs
hereditary spherocytosis - +FHx, neg coombs
both have predominantly extravascular hemolysis

50
Q

undifferentiated head & neck carcinoma risk facs?

A

smoking and EBV