March 14 - Heme/onc Flashcards

1
Q

Haldane effect

A

O2 affects affinity of hemoglobin for CO2/H+. Serves to increase CO2 delivery to lungs. Increase in PO2 increases CO2 and H+ offloading from Hb.

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2
Q

LMWH compared with UH

A
Better bioavailability
Can give SC
Less monitoring
Longer half half
Less easily reversible
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3
Q

Direct thrombin inhibitors

A

Argatroban, dabigatran, bivalirudin

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4
Q

Direct factor Xa inhibitors

A

Apixaban, rivaroxaban. Oral, don’t require monitoing but not as easily reversible.

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5
Q

Fondaparinux

A

Factor Xa inhibitor

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6
Q

Antiemetics used for chemo-induced vomiting

A

1) 5-HT3 antagonists: ondansetron and granisetron
2) Dopamine antagonists: metoclopromide, prochlorperazine
3) Neurokinin1 antagonists which prevent substance P release: aprepitant, fosaprepitant

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7
Q

Mutation in polycythemia vera (specifics)

A

Mutation in JAK2, which is involved in EPO signaling.

Normal pathway: EPO binds receptor. Receptor interacts with JAK2, which is a cytoplasmic tyrosine kinase receptor. Leads to downstream signling and activation of STATs.

Mutated pathway: JAK2 constitutively active resulting in overproduction of red cell.

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8
Q

Labs in sickle cell trait

A

Normal retic count, HGB, RBC indices and morphology

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9
Q

Integrins

A

Bind collagen, laminin, and fibronectin in the ECM to facilitate adhesion

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10
Q

Acute intermittent porphyria: presentation, pathophysiology, prevention, treatment of acute attacks

A

Presentation: episodes of abdominal pain, N/V, peripheral neuropathy, neuropsych derancements, red urine that darkens on exposure to light and air due to exccess PBG

Pathophys: AD defect in porphobilinogen deaminase in heme synthesis pathway. Causes early heme intermediates to accumulate and cause NS damage.

Prevention: avoid alcohol, smoking, and CYP inducing drugs which increase ALA synthase activity

Treatment : dextrose and IV heme which decrease ALA synthase activity

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11
Q

Cutaneous porphyrias

A

Caused by defect later in pathway than AIP. Photosensitivity

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12
Q

Heme synthesis pathway

A
Glycine and succinyl coA 
ALA
Porphobiolinogen
many steps
Porphyrin IX
Heme
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13
Q

Osteoblastic vs osteolytic bone mets: which cancers

A

Osteoblastic: prostate, small cell lung, hodgkin
Osteolytic: multiple myeloma, non-small cell lung, non-hodkin lymphoma, RCC, melanoma

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14
Q

Congenital parvovirus B infection, type of virus

A

Mom: arthritis
Fetus: hydrops fetalis due to interruption of erythropoiesis in bone marrow
Virus type: ssDNA

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15
Q

Congenital infections causing chorioretinitis

A

CMV or toxoplasma

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16
Q

Congenital infections causing sensorineural hearing loss

A

Rubella, CMV

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17
Q

Presentation of follicular lymphoma

A

Indolent, waxing and waning course. Painles LAD

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18
Q

Auer rods

A

Indicate myeloid differentation. Stain for peroxidase

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19
Q

RhoGAM: MOA

A

Anti-Rh(D) IgG pooled from donor plasma (polyclonal antibody). Binds to Rh-pos fetal RBCs that enter maternal circulation to prevent them from interacting with the maternal immune system and being sequestered in the spleen. Amount given is small enough that there is no significant transplacental fetal hemolysis.

IgG is given because that is what is produced against Rh antigens (vs IgM made against ABO)

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20
Q

Stop codons

A

UAA, UAG, UGA

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21
Q

Sickle cell mutation

A

Valine sub for glutamic acid

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22
Q

HMP shunt: location, products, key enzymes

A

In cytoplasm.
Produces ribose 5-phosphate and NADPH
Enzymes: transaldolase and transketolase

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23
Q

Blood cells in EBV infectious mono

A

Infects B cells by binding CD21 receptor
CD8+ cells clonally expand to kill the virus-infected cells. Appear larger than usual with abundant cytoplasm, eccentrically placed nuclei, membrane that appears to adhere to neighboring cells

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24
Q

Cyanide poisoning: pathophys, presentation, treatment

A

Pathophysiology: binds iron-containing cytochrome enzymes, inhibiting aerobic metabolism

Presentation: flushing, increased RR and HR, headache

Antidote: nitrites which induce formation of methemoglobin. Methemoglobin has high affinity for cyanide and sequesters it in blood

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25
Q

Beta thalassemia: mutation

A

Multiple mutaitons that affect mRNA

26
Q

Abciximab

A

GPIIb/IIIa receptor antagonist. Used for acute coronary syndrome, especially if undergoing percutaneous intervention

27
Q

Aspirin OD treatment

A

Alkalinize urine with sodium bicarb

28
Q

ADP receptor inhibitors: names, MOA, kinetics

A

Names: clopidogrel, ticagrelor, ticlopidine

MOA: block ADP receptor preventing GIIb/IIIa expression

Kinetics: prodrugs with slow onset of action, with exception of ticagrelor

29
Q

Phosphodiesterase III inhibitors: names, effects

A

Names: cilostazol, dipyridamole

Effects: inhibit platelet aggregation, vasodilate

30
Q

Inflammatory carcinoma: presentation and pathophys

A

Presentation: itchy, generalized erythematous rash on breast with thick and coarse skin texture and breast edema. Underlying mass may or may not be present.

Pathophys: cancer cells obstructing lymphatic drainage

31
Q

Cryoprecipitate vs fresh frozen palsma

A

FFP has all coag factors; cryoprecipitate only has cold-soluble proteins

32
Q

5-fluorouracil: MOA

A

pyrimidine analog that inhibits DNA synthesis by inhibiting thymidylate synthetase

33
Q

Leucovorin: MOA and effects

A

Tetrahydrofolate derivative that doesn’t require dihydrofolate reductase. USed to rescue normal cells from methotrexate. Can potentiate effects of 5-fluorouracil which uses THF as cofactor

34
Q

Vinca alkyloids: MOA and toxicity

A

MOA: M phase specific, inhibit microtubule formation
Toxicity: peripheral neuropathy

35
Q

G2 phase cancer drugs

A

Bleomycin and doxorubicin

36
Q

Why mature RBCs can’t make heme

A

Heme occurs partly in cytoplasm, partly in mitochondria. Mautre red cells have lost their mitochondria

37
Q

Warfarin induced skin necrosis

A

Complication seenin first few days of warfarin therapy due to transident hypercoagulable state

38
Q

Hairy cell leukemia: presentation and diagnosis

A

Presentation: pancytopenia and massive splenomegaly in middle aged male

Diagnosis: often a dry tap due to bone marrow fibrosis, massive splenomegaly due to cancer in the reticuloendothelial system as well as the marrow

39
Q

Ringed sideroblasts

A

Seen in myelodysplastic syndrome due to mitochondrial iron accumulaiton around the nucleus

40
Q

Tumor lysis syndrome

A

Emergency seen during chemo treatment of leukemia/lymphoma. Destroy a ton of cells at once resulting in release of ions and nucleic acids into the blood. Prevent with aggressive hdyration and medications.

41
Q

Rasburicase

A

Recombinant urate oxidase. Promotes conversino of uric acid to soluble metabolites that are excreted in urine.

42
Q

Translocation seen in burkitt lymphoma

A

t(8;14), c myc and IgH

43
Q

desmopressin: two uses

A
  1. Hemophilia A and von willebrand disease: increases factor VIII and vWF to stop bleeding
  2. Central DI: binds V2 receptors on renal tubular cells, increasing water reabsorption and decreasing urine production
44
Q

Leukemoid reaction

A

Benign leukocytosis in response to severe infection, malignancy, acute hemolysis. Presents with normal to elevates leukocyte alk phosphatase and basophilic granules in neutrophils on smear

45
Q

Myoglobin O2 binding curve

A

Monomeric without cooperative binding. Saturates at much lwoer pO2 than HGB (higher O2 affinity)

46
Q

Basis for hemoglobin electrophoresis: looking for HbS and HbC

A

Distinguishes based on charge. HbA has neg charged glutamate, HbS has nonpolar valine. HbC has pos charged lysine. Thus, HbA migrates farthest, HbC migrates the least toward the pos end of gel.

47
Q

6-mercaptopurine

A

Inactivated by xanthine oxidase. Azathioprine is pro-drug

48
Q

Distinguishing three types of childhood brain tumors

A
  1. Pilocytic astrocytoma: low grade, usually in cerebellum, presents with headache and balance problems, solid and cystic components on imaging, rosenthal fibers on path
  2. Medulloblastoma: high grade, always in cerebellum, solid mass, sheets of small blue cells
  3. Ependymoma: obstructive hydrocephalus, rosettes
49
Q

N-myc: what associated with

A

neuroblastoma, small cell lung cancer

50
Q

Bleomycin MOA

A

Forms radicals that damage DNA and produce double strand breaks; acts in G2

51
Q

Thyroid cancer with psammoma bodies

A

papillary carcinoma

52
Q

cancer associated with myasthenic syndrome

A

small cell lung cancer

53
Q

cancer associated with paraneoplastic hypercalcemia

A

squamous cell carcinoma

54
Q

cisplatin toxicity

A

Nephrotoxicity, acoustic nerve damage. Prevent nephrotoxicity with aggressive hydration

55
Q

von hippel lindau disease: cause and manifestationa

A

Caused by AD mutation on chromosome 3

Causes hemangioblastomas of retina, cerebellum, medulla; renal cell carcinoma

56
Q

Meningioma vs ependymoma (path and population(

A

Meningioma: psammoma bodies, middle aged woman

Ependymoma: perivascular pseeudorosettes, kids

57
Q

Sacrococcygeal teratoma

A

Most common fetal neoplasm, benign, primitive streak remnant

58
Q

Wilms tumor

A

Arises from embryonic renal cells due to WT del on chromosome 11. Presents as large palpable flank mass that distorts renal calyces

59
Q

Doxorubicin MOA

A

DNA intercalater

60
Q

Raltegravir MOA

A

integrase inhibitor for HIV

61
Q

DIC in pregnancy

A

mediated by tissue factor release from placental trophoblasts

62
Q

Metalloproteinases

A

enzymes that degrade ECM and basement membrane