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BOARDS specific notecards > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q
A
  • Acanthocyte (“spur cell”)
  • Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)
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2
Q
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  • Basophilic stippling
  • Lead poisoning, sideroblastic anemias, myelodysplastic syndrome
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3
Q
A
  • Dacrocyte (“teardrop cell”)
  • Bone marrow infiltration
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4
Q
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  • Degmacyte (“bite cell”)
  • G6PD deficiency
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5
Q
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  • Echinocyte (“burr cell”)
  • End-stage renal disease, liver disease, pyruvate kinase deficiency
  • Different from acanthocyte: projections more uniform and smaller
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6
Q
A
  • Elliptocyte
  • Hereditary elliptocytosis, usually asymptomatic
  • caused by mutation in genes encoding RBC membrane proteins (eg, spectrin)
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7
Q
A
  • Macro-ovalocyte
  • Megaloblastic anemia (also hypersegmented PMNs)
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8
Q
A
  • Ringed sideroblast
  • Sideroblastic anemia; Excess iron in mitochondria
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9
Q
A
  • Schistocyte
  • Microangiopathic hemolytic anemias, including DIC, TTP/ HUS, HELLP syndrome, mechanical hemolysis (eg, heart valve prosthesis)
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10
Q
A
  • Sicklecell
  • Sickling occurs with dehydration, deoxygenation, and at high altitude
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11
Q
A
  • Spherocyte
  • Hereditary spherocytosis, drug- and infection-induced hemolytic anemia
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12
Q
A
  • Target cell
  • HbC disease, Asplenia, Liver disease, Thalassemia (HALT the target)
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13
Q
A
  • Heinz bodies
  • Seen in G6PD de ciency.
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14
Q
A
  • Howell-Jolly bodies; Basophilic nuclear remnants found in RBCs
  • Seen in patients with functional hyposplenia or asplenia
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15
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19
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20
Q

Anti-coag factors produced by endothelium

A

PGI2 and NO

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21
Q

Ristocetin

A

Activates vWF to bind GpIb
Failure of aggregation in vW disease and Bernard-Soulier

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22
Q

α-thalassemia

A
  • 4 deleted: no α-globin; Excess γ-globin forms γ4 (Hb Barts): hydrops fetalis
  • 3 allele deletion: very little α-globin. Excess β-globin forms β4 (HbH)
  • 2 allele deletion: less clinically severe anemia
  • 1 allele deletion: no anemia (clinically silent)
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23
Q

β-thalassemia minor

A

β chain is underproduced; Usually asymptomatic.

Diagnosis confirmed by HbA2 (> 3.5%) on electrophoresis

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24
Q

β-thalassemia major

A

β chain is absent severe microcytic, hypochromic anemia with target cells and increased anisopoikilocytosis

Marrow expansion, B19 infxn

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25
Q

Lead poisoning

A
  • inhibits ferrochelatase and ALA dehydratase
    • decr heme and incr protoporphyrin

Symptoms of LEAD poisoning:

  • Lead Lines on gingivae (Burton lines) and on metaphyses of long bones D on x-ray.
  • Encephalopathy and Erythrocyte basophilic stippling
  • Abdominal colic and sideroblastic Anemia
  • Drops—wrist and foot drop
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26
Q

Treatment for lead poinsoning

A

Dimercaprol and EDTA are 1st line of treatment

Succimer used for chelation for kids

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27
Q

Sideroblastic anemia causes

A

X-linked defect in ALA synthase gene)

myelodysplastic syndromes

reversible: (alcohol is most common; also lead, vitamin B6 de ciency, copper de ciency, isoniazid)

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28
Q

Siderblastic anemia lab findings

A

Incr iron, decr TIBC, incr ferritin

Ringed siderblasts

Basophilic stippling

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29
Q

Treatment for sideroblastic anemia

A

pyridoxine (B6, cofactor for ALA synthase).

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30
Q

Folate deficiency

A

Malnutrition, malabsorption, drugs

Incr homocystein; nml methylmalonic acid

31
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Vit B12 deficiency

A

Vegetarian, Crohn, tapeworm

Incr homocystein and methylmalonic acid

SHilling test +

32
Q

Orotic aciduria

A

Inability to convert orotic acid to UMP
(de novo pyrimidine synthesis) d/t defect in UMP synthase

failure to thrive, developmental delay, and megaloblastic anemia refractory to folate and B12

33
Q

Diamond-Blackfan anemia

A

Rapid-onset anemia within 1st year of life due to intrinsic defect in erythroid progenitor cells.

34
Q

Hereditary spherocytosis

A

Extravascular hemolysis; defect in RBC membrane protins (ankyrin)

Small, round RBCs with less surface area and no central pallor ( MCHC)

+osmotic fagility test

35
Q

G6PD defeciency

A

Decr glutathione: increased susecptibility to oxidative stress

Blood smear shows RBCs with Heinz bodies and bite cells

36
Q

Pyruvate kinase defeciency

A

Decreased ATP -> rigid RBCs

Increased 2,3 BPG -> decr Hg affinity for O2

37
Q

Paroxysmal nocturnal hemoglobinuria

A

Increased complement destruction of RBC; impaired synthesis of GPI anchor for decay-accelerating factor

38
Q

Warm hemolytic anemia

A

IgG, seen in SLE and CLL

39
Q

Cold hemolytic anemia

A

IgM

CLL, mycoplasma, IM

40
Q

Acute intermittent porphyria

A

Decr Porphobilinogen deaminase; Porphobilinogen, ALA accumulate

41
Q

Porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase deficiency

Uroporphyrin (tea- colored urine) builds up

42
Q

Iron poisoning

A

Cell death due to peroxidation of membrane lipids

43
Q

t(8;14)

A

Burkitt lymphoma (c-myc activation)

44
Q

t(9;22)

A

Philadelphia

(BCR-ABL hybrid)

45
Q

t(11;14)

A

Mantle cell lymphoma (cyclin D1 activation)

46
Q

t(14;18)

A

Follicular lymphoma (BCL-2 activation)

47
Q

t(15;17)

A

APL (M3 type of AML)

48
Q

Busulfan

A

Cross-links DNA

Treat CML

SE: myelosuppression, pulmonary fibrosis, hyperpigmentation

49
Q

Bevacizumab

A

MCA against VEGF; Inhibits angiogenesis

Treats Solid tumors

SE: Hemorrhage, blood clots, and impaired wound healing.

50
Q

Erlotinib

A

EGFR tyrosine kinase inhibitor.

Non-small cell lung carcinoma.

Rash.

51
Q

Cetuximab

A

Monoclonal antibody against EGFR.
Stage IV colorectal cancer (wild-type KRAS), head and neck cancer.

Rash, elevated LFTs, diarrhea.

52
Q

Imatinib

A

Tyrosine kinase inhibitor of BCR-ABL (Philadelphia chromosome fusion gene in CML) and c-kit (common in GI stromal tumors).

CML, GI stromal tumors (GIST)

Fluid retention.

53
Q

Rituximab

A

Monoclonal antibody against CD20, which is found on most B-cell neoplasms.

Non-Hodgkin lymphoma, CLL, ITP, rheumatoid arthritis.
Incr risk of progressive multifocal leukoencephalopathy.

54
Q

Bortezomib, carfillzomib

A

Proteasome inhibitors, induce arrest at G2-M phase and apoptosis. Multiple myeloma, mantle cell lymphoma.
Peripheral neuropathy, herpes zoster reactivation.

55
Q

Tamoxifen, raloxifene

A

Selective estrogen receptor modulators (SERMs)—r- antagonists in breast and agonists in bone: Block binding of estrogen to ER ⊕ cells.

Breast cancer (tamoxifen only) and prevention

Raloxifene prevents osteoporosis.

Tamoxifen—partial agonist in endometrium: incr risk of endometrial cancer; “hot ashes.” Raloxifene—no risk of endometrial carcinoma (so you can relax!):estrogen receptor antagonist in endometrial tissue.
Both risk of thromboembolic events (eg, DVT, PE).

56
Q

Trastuzumab (Herceptin)

A

Monoclonal antibody against HER-2 (c-erbB2), a tyrosine kinase receptor: inhibition of HER2-initiated cellular signaling and antibody- dependent cytotoxicity.

HER-2 ⊕ breast cancer and gastric cancer (tras2zumab). Cardiotoxicity.

“Heartceptin” damages the heart.

57
Q

Vemurafenib

A

Small molecule inhibitor of BRAF oncogene ⊕ melanoma. VEmuRAF-enib is for V600E- mutated BRAF inhibition.

Metastatic melanoma.

58
Q

Rasburicase

A

Recombinant uricase that catalyzes metabolism of uric acid to allantoin

Prevention and treatment of tumor lysis syndrome.

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BOARDS specific notecards (42 decks)

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