UWorld- Heme/Onc Flashcards

taken 3/10/14

1
Q

what kind of heparin (unfractionated, low molecular weight like enoxaparin or dalteparin, fondaparinux) is most effective at inactivating thrombin

A

unfractionated heparin; it has a long enough polysaccharide chain to form a ternary complex with antithrombin and thrombin to inactivate thrombin

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2
Q

what kind of drugs are argatroban and bivalirudin and what effect do they have on PT, aPTT and thrombin time (TT)

A

direct thrombin inhibitors (derived from hirudin from leeches);
they increase thrombin time, but have little effect on PT or aPTT

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3
Q

if a patient has a t(15;17) translocation what treatment would you provide?
what’s the disease?

A

treat with all-trans-retinoic acid (ATRA)

this patient has APML (which involves a defective retinoic acid receptor)

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4
Q

a patient for whom Heinz bodies are seen on blood smear and who tends to experience jaundice and dark urine after taking antimalarials and sulfonamides has inherited his/her disease via what inheritance pattern

A

G6PD deficiency is X-linked

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5
Q

nitrite poisoning will cause what to happen to PO2, % Hb sat, and O2 content

A

methemoglobinemia, caused by nitrite, which oxidize Fe2+ to ferric oxide (Fe3+) leads to:

  • normal PO2 (amt. of O2 dissolved in the plasma is unchanged)
  • decreased Hb saturation
  • decreased O2 content
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6
Q

A patient with sore throat, malaise, persistent fever, cervical LAD, myalgias and splenomegaly has atypical cells on blood smear that have an eccentrically placed nucleus, ample cytoplasm and edges that seem to take the shape of the cells around them. What are these atypical cells?
What is the likely disease?

A

atypical CD8+ cytotoxic lymphocytes

EBV

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7
Q

A patient is on warfarin therapy after an acute cardiac incident. She says she’s taking her medications, but her PT is subtherapeutic. What other drugs might this patient be taking?

A

CYP450 inducers increase warfarin metabolism (removal)

ethosuximide, barbituates, phenytoin, chronic alcohol and smoking, St. John’s wort, glucocorticoids, carbamazepine, rifampin, griseofulvin

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8
Q

what kind of heparin is enoxaparin and how does it work

A

enoxaparin is a low molecular weight heparin that binds antithrombin to activate it, leading to inactivation of Factor Xa (preventing cleavage of prothrombin to thrombin)

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9
Q

A 5 year old had abdominal pain, diarrhea and fever. A few days later he presents with pallor and oliguria. Which lab findings would be decreased? Increased?

A

This patient probably has HUS.

increased: bleeding time, retic, LDH, BUN, Cr
decreased: platelets, Hgb, Hct, RBC count

note: PT and PTT are normal b/c no coag factor involvement

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10
Q

what are Nef and Tat? explain what they do.

A

Nef and Tat are HIV genes; Nef decreases MHC I expression on infected cell surfaces; Tat plays a role in viral replication

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11
Q

a patient with recurrent respiratory and GI infections is found to have low serum immunoglobulin;
what substructure in the lymph node is he/she likely lacking and what disease does this patient likely have

A

lacking germinal centers;

this patient has Bruton X-linked agammaglobulinemia which causes defective B cell maturation

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12
Q

what values are best for diagnosing/ confirming suspicion of hereditary spherocytosis

A

osmotic fragility test will indicate fragility of the membrane, characteristic of HS
also, mean corpuscular hemoglobin concentration will be increased due to membrane loss and dehydration of the cell

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13
Q

where is major basic protein found and what does it do

A

found in eosinophilic granules and protects against parasites

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14
Q

myeloperoxidase stains for what liquid cancer

A

AML

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15
Q

what is argatroban; name the drug type and others of its class

A

argatroban, along with hirudin and lepirudin, are direct thrombin inhibitors (they inhibit thrombin, but don’t affect factor Xa and they are used for anticoagulation as an alternative to heparin for patients with HIT)

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16
Q

explain the relative locations of HbA, HbH, HbC and HbS on hemoglobin electrophoresis

A

HbH moves farthest (tetramers of normal beta globin); then HbA (negative a.a. residues); then HbS (a valine aka neutral a.a. substitution); HbC moves the shortest distance (a lysine aka positive a.a. substitution)

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17
Q

what are the 5 steps of leukocyte accumulation in the tissues

A
  1. margination: vascular leakage allows leukocytes to contact endothelium
  2. rolling: selectins mediate loose binding to endothelium
  3. activation: while rolling neutrophils sample chemokines that activate integrins
  4. tight adhesion and crawling: integrins bound to ICAM promote tighter adhesion
  5. transmigration: in the periphery integrins and PECAM-1 promote extravasation into interstitium
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18
Q

what is the preferred route of iron administration

A

oral iron supplementation (parenteral iron is only given to patients who cannot take orally or need iron fast)

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19
Q

are most cases of B12 deficiency treated with oral or parenteral administration

A

parenteral: most cases of vit B12 deficiency are due to malabsorption so oral supplementation would be inadequate

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20
Q

the three symptoms eczema, thrombocytopenia and combine B and T cell deficiency (manifested as repeated infections) suggest what disease

A

Wiskott-Aldrich syndrome

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21
Q

why is vitamin K deficiency seen in preterm infants and what disease does it cause

A

preterm infants may have vitamin K deficiency due to immature liver, low vit K content in breast milk and meager intestinal microbiota;
vit K deficiency can cause hemorrhagic disease of the newborn due to low factors 2, 7, 9, and 10

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22
Q

name the chromosomes where the following genes are found:

bcl-2, c-myc, Ig heavy chain, kappa light chain, lambda light chain

A
bcl-2 = chromosome 18
c-myc = chromosome 8
Immunoglobulin heavy chain = chromosome 14
kappa light chain = chromosome 2
lambda light chain = chromosome 22
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23
Q

what is ticlopidine and what is a deadly, but rare side effect of its use

A

ticlopidine is an ADP antagonist (along with clopidogrel) and in rare cases it causes neutropenia (typically presenting with fever and mouth ulcers)

24
Q

what are the most common ways to contract enterohemorrhagic E.coli

A
  1. undercooked beef
  2. person-to-person contact
  3. raw unpasteurized milk
25
Q

gp41 mediates viral _________ while gp120 mediates viral __________

A

gp41 mediates viral entry while gp120 mediates viral attachment

26
Q

what conditions make HbS RBC sickling more likely to occur

A

low oxygen levels, low volume (dehydration) and acidic environment

27
Q

what molecular interaction allows fetal hemoglobin to have higher O2 affinity

A

impaired 2,3-DPG binding (2,3,-DPG effectively hinders O2 affinity)

28
Q

if a cancer patient develops peaked T waves, elevated BUN/Cr, hypocalcemia and/or hyperphosphatemia what underlying condition would you suspect and how could it have been prevented

A

tumor lysis syndrome presents as hyperkalemia, hyperuricemia causing renal dysfunction, hyperphosphatemia and hypocalcemia

treat chemo patients with allopurinol, febuxostat, or rasburicase

29
Q

differentiate Hodgkin’s from non-Hodgkin’s lymphoma

A

Hodgkin’s has Reed-Sternberg cells (giant bilobed or binucleate cells), better prognosis, more constitutional symptoms and is more localized to a single group of nodes

30
Q

pol and env mutations in HIV allow for what modes of resistance, respectively

A

pol mutations allow for resistance to reverse transcriptase inhibitors

env mutations allow for resistance to host neutralizing antibodies

31
Q

what CBC abnormalities would you expect for polycythemia vera and what is mutated to cause this condition

A

polycythemia, thrombocytosis, leukocytosis;

JAK2 mutation causes hyperproliferation of all three myeloid cell lines

32
Q

bone marrow biopsy showing greater than 30% plasma cells is diagnostic of what condition

A

multiple myeloma

33
Q

what does ristocetin do

A

ristocetin is used to test for vWF abnormalities;

ristocetin activates GP1b and so in normal blood adding ristocetin will promote increased platelet aggregation

34
Q

in Hemophilia A and B patients, administration of what factor can allow for clotting

A

thrombin

35
Q

in what conditions or cases would you see burr cells?

what are burr cells?

A

burr cells are RBCs with evenly spaced spikes;
seen as an artifact sometimes, but seen characteristically in PKD, microangiopathic hemolytic anemia, uremia and mechanical trauma

36
Q

describe the effect of selective COX-2 inhibitors on inflammation, gastric mucosa and platelet aggregation

A

anti-inflammatory
no gastric mucosal disturbances as seen in NSAIDs
no disruption of platelet aggregation since platelets express mainly COX-1

37
Q

explain the Haldane effect

A

as deoxygenated blood enters the alveolar capillaries, increased PO2 leads to increasing binding of oxygen to hemoglobin and consequent release of H+ and CO2 from hemoglobin

38
Q

what gives reticulocytes their bluish color on Wright-Giemsa stain

A

ribosomal RNA that has not yet disappeared

39
Q

what kind of drug is raltegravir and what does it treat

A

raltegravir is an HIV integrase inhibitor

40
Q

which drugs are used for clot lysis in acute MI

A

fibrinolytics such as t-PA, alteplase, tenecteplase and recteplase

41
Q

describe the mechanism by which paroxysmal nocturnal hemoglobinuria develops

A

deficiency of CD55 and 59 leads to inability to inactivate complement –> hemolysis that leads to hemoglobinuria

42
Q

what are the three most common symptoms of paroxysmal nocturnal hemoglobinuria

A

hemolysis (intravascular), hypercoagulability, and pancytopenia

43
Q

pure red cell aplasia commonly results from what underlying illnesses

A

thymomas and parvovirus B19 infection (because the virus preferentially infects proerythroblasts)

44
Q

how does warfarin’s effects on vitamin K lead to down regulation of clotting factors

A

warfarin prevents vitamin K-mediated carboxylation of glutamate residues on factors 2, 7, 9, 10, Protein C and S

45
Q

in aplastic anemia what replaces hematopoetic tissue in the bone marrow

A

fat cells and fibrous stroma

46
Q

rat poison contains derivatives of 4-hydroxycoumarin; how does rat poison cause death

A

4-hydroxycoumarin is a vitamin-K antagonist

47
Q

what is the primary cause of iron deficiency anemia

A

blood loss

48
Q

why does myelofibrosis cause hepatosplenomegaly

A

decreased production of blood cells in the bone marrow leads to extramedullary hematopoesis

49
Q

what defect causes myelofibrosis

A

a mutation causing valine to phenylalanine substitution in the JAK-2 kinase (a tyrosine kinase) leads to constitutive activation of the JAK-STAT pathway

50
Q

what drug treats myelofibrosis and how

A

ruxolitinib is a JAK2 inhibitor

51
Q

over what time period in childhood is fetal hemoglobin gradually replaced by adult hemoglobin

A

first 6 months of life

52
Q

which anticoagulant is used to treat DVT in pregnant women

A

heparin

53
Q

differentiate the location of positive and negative T-cell selection and when they occur in relation to each other

A

positive selection occurs first and takes place in the thymic cortex;
negative selection occurs after positive selection and takes place in the thymic medulla

54
Q

what kind of blood problem does uremia cause

A

qualitative platelet disorder (note: platelet count is normal)

55
Q

how does the JAK2 mutations seen in myeloproliferative disorders (except for CML) cause myeloproliferation

A

increased bone marrow sensitivity to growth factors