Heme - Onc

Question 1

Intrinsic coagulation pathway

Answer 1

XII, XI, IX, VIII (IIa), —> X, II, Fibrinogen/fibrin, XIII

Reflected in PTT

Question 2

Extrinsic coagulation pathway

Answer 2

VII –> X, II, Fibrinogen/fibrin, XIII

Reflected in PT

Question 3

VitK coag factors

Answer 3

II, VII, IX, X

Anti: C, S

Question 4

Anticoagulation

Answer 4

Protein C –> C* –S–> cleaves Va, VIIIa

Plasminogen –tPA–> plasmin –> cleave fibrin, destroy coag factors

Question 5

Factor V Leiden

Answer 5

resists inhibition by protein C.

Question 6

Heparin

Answer 6

Enhances antithrombin (cleave II, Xa).

Unfractionated: II, Xa

LMWH: Xa

Question 7

vWF

Answer 7

binds GpIb (absence of = Bernard-Soulier syndrome).

Activated by ristocetin (no change in resposne in vW disease).

Question 8

ADP receptor

Answer 8

Leads to increased GpIIb/IIIa on surface.

Blocked by clopidogrel, ticlopidine (AE neutropenia).

Question 9

GpIIb/IIIa

Answer 9

Binds fibrinogen to crosslink plts.

Deficiency: glanzmann thrombasthenia.

Blocked by abciximab.

Question 10

Paroxysmal nocturnal hemaglobinuria

Answer 10

CD55 and CD59 are absent.
Normally, they inactivate complement, thus complement-mediated hemolysis.

Tx: eculizumab.

Question 11

Lead poisoning

Answer 11

Enzyme: ferrochelatase, ALA dehydratase.

Accumulated: protoporphyrin, delta-ALA

Sx: microcytic anemia, GI and kidney disease. Children (mental deterioration). Adults (HA, memory loss, demyelination).

Question 12

Acute intermittent porphyria

Answer 12

Enzyme: Porphobilinogen deaminase.

Accumulate: porphobilinogen, delta-ALA, coporphobilinogen (urine).

Sx: painful abdomen, port wine-colored urine, polyneuropathy, psychological disturbances, precipitated by drugs, alcohol, starvation.

No cutaneous manifestation.

Tx: glucose, heme.

Question 13

Porphyria cutanea tarda

Answer 13

Enzyme: uroporphyrinogen decarboxylase.

Accumulate: uroporphyrin (uroporphyrinogen III).

Sx: blistering cutaneous photosensitivity.

Most common porphyria.

Question 14

Alkaline phosphatase (CML vs Leukamoid)

Answer 14

Leukamoid: increased.

CML: decreased.

Question 15

Reed-Sternberg cells:

-Surface proteins

Answer 15

CD15, CD30

Question 16

Diffuse large B-cell lymphoma (translocation)

Answer 16

t(14;18)

Question 17

Mantle cell lymphoma (translocation)

Answer 17

t(11;14) cyclin D1 to heavy-chain.

CD5+

Question 18

Folicular lymphoma (translocation)

Answer 18

t(14;18) heavy-chain and bcl-2

Indolent course. Waxing and waining.

Question 19

ALL

Answer 19

Children. Associated with down syndrome.

TdT+

Precursor B cell type: CD 10+, CD 19+
Precursor T cell type: CD 2, 3, 4, 5, 7, 8

t(12;21) = better prognosis.

Question 20

SLL / CLL

Answer 20

Elderly.

CD20+, CD5+, B cell neoplasm.

Smudge cells!

Slow and often asymptomatic.

Question 21

Hairy Cell leukemia

Answer 21

Adults.

Stains: tartrate-resistant acid phosphatase (TRAP).

Marrow fibrosis –> dry tap.

Tx: cladribine (2-CDA) = adenosine analog.

Question 22

AML

Answer 22

Elderly.

t(15;17) tx retinoic acid.

Auer rods = peroxidase (esp. AML M3).

Risks: alkylating chemo, myeloproliferative disorders, downs.

DIC possible s/p chemo 2/2 auer rod release.

Question 23

CML

Answer 23

45-85 y/o.

t(9;22) bcr-able.

Increased neutrophils, metamyelocytes, basophils. Splenomegaly.

Can –> AML or ALL (blast crisis).

Low LAP.

Rx: imatinib.

Question 24

P450 inhibitors

Answer 24

Increase warfarin

Cimetidine
Bactrim
Amiodarone
Metronidazole

Question 25

P450 inducers

Answer 25

Decrease warfarin

Phenytoin
Phenobarbital
Rifampicin

Question 26

Give Doxorubicin with

Answer 26

Dexrazoxane

Question 27

Give cisplatin/carboplatin with

Answer 27

amifostine

Question 28

Give cyclophosphamide with

Answer 28

mesna

Question 29

Give methotrexate with

Answer 29

leucovorin = folinic acid