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COMLEX > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

hypochromic, microcytic anemia

  • low iron, low ferretin
  • high TIBC

koilonychia, atrophic glossitis, weakness, pallor

A

iron def anemia

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2
Q

African American, self-limiting fatigue and jaundice after taking NSAIDs or TMP-SMX

  • dark urine, increased bili
  • AR
A

G6PD deficiency

- hemolysis after oxidative stress

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3
Q

warfarin effect on PT, PTT, bleed time

A

PT: up
PTT: +/-
bleed time: normal

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4
Q

heparin effect on PT, PTT, bleed time

A

PT: normal
PTT: up*
bleed time: normal

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5
Q

vWD effect on PT, PTT, bleed time

A

PT: normal
PTT: normal
bleed time: up

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6
Q

DIC effect on PT, PTT, bleed time

A

PT: up
PTT: up
bleed time: up

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7
Q

hemophilia A effect on PT, PTT, bleed time

A

PT: normal
PTT: up
bleed time: up

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8
Q

young female, mediastinal mass, painless LAD, B sx, Reed Sternberg, lacunar cells, lymphocytes

A

HL: nodular sclerosis type

- excellent prog

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9
Q

t(14:18)

A

follicular lymphoma

  • second most common B cell lymphoma
  • arises from germinal centers
  • middle age caucasians
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10
Q

t(8:14)

A

Burkitt lymphoma

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11
Q

auer rods?

A

APML

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12
Q

t(15:17)

A

APML

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13
Q

hypotension, paresthesias, paralysis caused by eating fish

A

tetrodotoxin, from puffer fish

- blocks sodium channels

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14
Q

what is the MOA of latrotoxin (black widow spider)?

A

depolarizes neurons -> enhances calcium -> uncontrolled exocytosis from nerve terminals

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15
Q

type I collagen defect arising from a deficiency of the amino acid glycine

  • blue sclera
  • frequent fractures
  • scoliosis, hyperextensible joints
A

osteogenesis imperfecta

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16
Q

type III and V collagen defect

  • hypermobile joints, tendon rupture, fragile skin
  • dental crowding, flat feet
  • valvular heart disease (MVP)
  • myalgias
A

Ehlers-Danlos

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17
Q

MC lymphoma of adulthood, males in their 80’s

  • usually asx, or painless LAD
  • small mature lymphocytes, clumped chromatin, smudge cells
  • CD 5, CD 19, CD 20, CD 23
A

CLL

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18
Q

δ-aminolevulinic acid is formed by the combination of what?

A

succinyl-CoA and glycine

- first step in heme synthesis (inhibited by lead)

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19
Q

ristocetin cofactor assay is diagnostic of what?

A

vWD

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20
Q

what 2 enzymes does lead inhibit?

A
  1. ferrochelatase (last enzyme in the pathway of heme synthesis)
  2. δ-aminolevulinic acid dehydratase (ALA-dehydratase)
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21
Q

what is tx for mild hemophilia?

A

desmopressin

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22
Q

70-80’s, fever, night sweats, and weight loss

  • anorexia, fatigue, chest discomfort
  • mediastinal lymphadenopathy -> rapidly enlarging mass
  • extranodal manifestations are common in liver, spleen, testes, etc
  • on histo, diffuse infiltrate of large and small cells
  • xsome translocation involving BCL-6.
A

diffuse large B cell lymphoma

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23
Q

what 3 drugs are known to cause folate deficiency?

A
  1. methotrexate
  2. OCP’s
  3. phenytoin -> inhibits intestinal conjugase that converts polyglutamate to monoglutamate in the jejunum
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24
Q

what seizure med inhibits sodium influx into neurons, thereby decreasing their ability for depolarization

  • does so for neurons in the resting state and in the depolarization state
  • adverse drug reactions include gingival hyperplasia, megaloblastic anemia secondary to folate deficiency, hirsutism, and inhibition of anti-diuretic hormone release
A

phenytoin

- teratogenic

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25
Q

elevated homocysteine?

A

folate def

  • elevated levels of homocysteine -> hypercoagulability
  • risk of vessel thrombosis
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26
Q

elevated homocysteine and MMA?

A

vit B12 def

- look for neurological sx to distinguish folate from b12 def!

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27
Q

Anti-microsomal antibodies are associated with what?

A

Hashimotos thyroiditis

28
Q

Anti-histone antibodies are associated with what?

A

drug-induced SLE

29
Q

Antinuclear antibody (ANA) screening

  • specific tests for anti-topoisomerase I (anti-Scl-70), anticentromere (ACA)
  • anti-RNA polymerase III antibodies
A

scleroderma

30
Q

teenage male with mid shaft pain of long bones

  • bx shows anaplastic small blue cells with hyperchromatic nuclei and scant cytoplasm
  • “onion skin” layers around the involved diaphysis
  • MIC2 (12E7) antigen (CD99) positive
A

ewing sarcoma

- t(11:22)

31
Q

malignant tumor that peaks at 10 to 20 years of age

  • arises from the metaphysis of long bones
  • destruction of the marrow cavity, and extension into the surrounding tissue with formation of a Codman triangle
  • sunburst pattern on x-ray
A

osteosarcoma

32
Q

rare, slow-growing, locally aggressive neoplasms of bone that arise from embryonic remnants of the notochord

  • tumors typically occur in the axial skeleton and are most common in the inner skull base and sacrum (will never be in extremities)
  • vesicular nuclei with abundant vacuolated, soap bubble-like cytoplasm and contain glycogen or mesenchymal mucin
A

chordoma

33
Q

individuals between 20 and 40 years of age with pain, swelling, and limited joint movement

  • tumor in the epiphyseal end of long bones (distal femur or proximal tibia)
  • double bubble or soap-bubble on x-ray
  • sheets of round to oval polygonal or spindle-elongated mononuclear cells that are interspersed with multinucleated osteoclast-like giant cells
A

giant cell tumor

34
Q

men 30-60 years old

  • usually located in the pelvis, spine, scapula, humerus, tibia, and femur
  • low to intermediate grade and show moderate cellularity with small, round nuclei; the cells are occasionally binucleated
  • abundant hyaline cartilage-type matrix
A

chondrosarcoma

35
Q

anti-Jo-1 antibodies are associated with what?

A

dermatomyositis

  • idiopathic inflammatory myopathy characterized by symmetric proximal skeletal muscle weakness and evidence of muscle inflammation
  • Heliotrope eruption (erythematous eruption on the upper eyelids)
  • Gottron papules (violaceous papules on the dorsal aspects of MCP/lP joints)
36
Q

C1 esterase deficiency?

A

hereditary angioedema

- episodes of angioedema and severe laryngeal edema that could be life-threatening

37
Q

CD40 ligand defect

A

hyper IgM

  • B cells can’t receive proper activation signal -> no class switching (lots of IgM, nothing else)
  • present with infections due to Pneumocystis jiroveci (fungi), cytomegalovirus, or Cryptococcus (fungi)
38
Q

Male infants usually present with prolonged bleeding, petechiae, purpura, or abnormal bruising

  • thrombocytopenia, eczema, and a humoral immunodeficiency due to decreased amounts of IgM
  • increased risk for skin disorders, such as impetigo, cellulitis, furuncles, or abscesses. Otitis media, sinus infections, pharyngitis, and thrush
A

Wiscott-Aldrich syndrome (x-linked)

- low IgM, high IgA, IgE

39
Q

X-linked disorder found primarily in males that presents early in childhood with recurrent infections with extracellular encapsulated bacteria
- patients will have an absence of B cells, however, normal T cell levels will be present

A

Bruton agammaglobulinemia

40
Q

don’t forget that impetigo can also lead to acute post strep glomerulonephritis!!

A

:)

41
Q

CD40L def?

A

hyper IgM, can’t class switch

42
Q

defect in DNA double-strand break repair enzymes is commonly associated with what?

A

ataxia-telangiectasia (Louis-Bar syndrome)

- can lead to ataxia, spider angiomas, and immunoglobulin (Ig) A deficiency

43
Q

Defective phagosome-lysosome fusion in neutrophils is seen in what?

A

Chediak-Higashi syndrome

- can present with recurrent pyogenic infections, along with partial albinism and peripheral neuropathy

44
Q

lack of nicotinamide adenine dinucleotide phosphate oxidase results in chronic granulomatous disease, which decreases reactive oxygen species leading to recurrent infection with what organisms?

A

catalase-positive organisms (Staphylococcus aureus, Pseudomonas aeruginosa, Klebsiella pneumoniae)

45
Q

competitive inhibitor of reverse transcriptase

- adverse effects include bone marrow suppression, hepatitis, and lactic acidosis

A

Zidovudine

46
Q

disorder characterized by the excretion of urine that appears normal when fresh but turns dark brown or black if left standing or after alkalinization

  • asx in childhood
  • during the third decade, deposits of the brownish or bluish pigment become apparent, typically first in the ear cartilage and sclerae
A

alkaptonuria
- the dark color is caused by oxidation of homogentisate, which is why alkaptonuria has also been called black urine disease

47
Q

complement protein required for neutrophil chemotaxis?

A

C5a

48
Q

failure to thrive

  • mental retardation
  • microcephaly
  • large facial bones
  • gapped teeth/enamel defects
  • eczematous rash
  • hyperreflexia/increased muscle tone
  • fair skin, urine with a “mousy” odor
A

PKU (AR)

  • no phenylalanine hydroxylase = can’t form tyrosine
  • must supplement tyrosine in diet!
49
Q

what antibiotic inhibits DNA gyrase to block replication

A

fluoroquinolones

50
Q

what TB med induces P450, thereby increasing metabolism of warfarin and decreasing international normalized ratio (INR) levels?

A

rifampin

- inhibits DNA-dependent RNA pol

51
Q

what is the tx for malignant hypothermia?

- developing muscle rigidity, hypertensive urgency, and increased end-tidal CO2

A

dantrolene

52
Q
  • elfin face
  • mental developmental delay
  • well-developed verbal skills
  • friendly to strangers
  • high calcium levels d/t increased sensitivity to vitamin D
A

Williams syndrome

- microdeletion on Xsome 7 (elastin gene)

53
Q

difference between FISH and karyotype?

A

FISH: Presence/absence of specific DNA sequence in a specific chromosome location
karyotype: gross abnormalities (trisomy)

54
Q
  • midfacial underdevelopment
  • microcephaly
  • delayed CNS development
  • dysmorphic facial features: smooth philtrum, thin vermillion (lip) border, and small palpebral fissures
  • height, weight, and head circumference likely at or below the 10% percentile (small for gestational age)
A

fetal alcohol syndrome

55
Q

delayed closure of fontanelles, parietal/frontal bossing, enlargement of the costochondral junction (rachitic rosary), widening of the wrist, and lateral bowing of the femur and tibia (genu varum)

A

rickets

56
Q

Askenazi jew with hypoglycemia, lactic acidemia (primary cause of the high anionic gap acidosis), hypertriglyceridemia, hyperuricemia, and hepatomegaly

A

von Gierke

  • Inactivating mutations in the gene encoding glucose-6-phosphatase
  • GSD type 1
57
Q

dark urine due to increased porphyrins or porphobilin

  • poorly localized abdominal pain (most common)
  • may be accompanied by diarrhea and decreased bowel sounds
  • decreased DTRs, tachycardia
A

acute intermittent porphyria (AD)

- defect in porphobilinogen (PBG) deaminase, an enzyme involved in heme synthesis

58
Q

mutation causing increased purine synthesis?

- phosphoribosyl pyrophosphate synthetase

A

can cause gout!

- NOTE: neutrophils are primary cells responsible for inflammatory rxn

59
Q

urase producing organisms that can precipitate struvite stones?

A

klebsiella, proteus

60
Q

hemolytic anemia, jaundice, splenomegaly

- pigmented gallstones

A

hereditary spherocytosis

61
Q

deficiency of homogentisic acid dioxygenase

- blue-black pigment builds up in connective tissue (sclera, ear cartilage)

A

alkaptnuria (AR)

62
Q

virulence factor of N. meningitidis?

A

lipooligosacharide endotoxin

  • release of endotoxin into blood causes rapid-onset sepsis
  • endotoxin is in gram-NEG only!
63
Q

virulence factor of S. pyogenes?

A

M protein

- prevents phagocytosis, inhibits complement binding, aids in epithelial attachment

64
Q

virulence factor of S. aureus?

A

Panton-Valentine leukocidin (VPL)

  • destroys leukocytes, causes tissue necrosis
  • only found in most virulent strains of S. aureus (MRSA) that cause skin and soft tissue abscesses and necrotizing pneumonia
65
Q

virulence factor of S. pneumoniae?

A

polysaccharide capsule

- prevents phagocytosis

66
Q

round budding yeast with thick capsule?

A

cryptocacus neoformans

- causes meningoencephalitis in immunocomp

COMLEX (14 decks)

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