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MALIGNANT HEME > SET6 > Flashcards

SET6 Flashcards

1
Q

What is the most favorable cytogenetic abnormality in CLL?

A

Deletion of 13q

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2
Q

Veno-occlusive disease due to transplant was previously called ______

A

Sinusoidal Obstruction Syndrome; US will show reversal of portal venous flow

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3
Q

What is high dose Daunorubicin? Intermediate dose? What patients may benefit from high dose daunorubicin?

A

90 mg/m2 as opposed to 60 (this is the 7+3 dosing); patients with DNTM3A, mutant NPM1, and MLL translocations may benefit

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4
Q

From worst to best list 5 cytogenetic abnormalities in CLL

A

17p del (32 months), 11q del (79 months), Normal Cytogenetics (114 months), trisomy 12 (111 months), 13q del (133 months)

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5
Q

What effect can Ibrutinib initially have on CLL lymphocyte counts? Why?

A

It can actually cause an increase because it, in addition to other things, inhibits binding of the CLL cells to the stroma

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6
Q

Which source of HSCs has the best platelet recovery?

A

Cord blood

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7
Q

What is the deal with Isocitrate Dehydrogenase mutations in AML with respect to prognosis?

A

IDH2 mutations associated with better prognosis; IDH1 is not

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8
Q

Deletion of 17p implies loss of what gene?

A

TP53 which is located on 17p so you’ve lost the “guardian of the genome”

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9
Q

What is the flow cytometry for APML?

A

HLADR-, CD34- (they are promyelocytes not blasts), MPO+, and CD33+

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10
Q

Why do the vast majority of Hairy Cell patients have splenomegaly?

A

Due to extramedullary hematopoeisis

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11
Q

Patients who are determined to be high risk for veno-occlusive disease with allo-HSCT should receive prophylactic _______

A

Defibrotide

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12
Q

What does CEBPA stand for? What is the relationship with AML?

A

CCAAT Enhancer Binding Protein Alpha; BIALLELIC mutations are associated with a better prognosis

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13
Q

What imaging would be useful if you suspected that a person was suffering from veno-occlusive disease due to acute GVHD from transplant?

A

Doppler US of liver will show reversal of portal venous flow

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14
Q

What is a good way to manage patients under 60 who have a late relapse from their initial AML?

A

They should be reinduced with a salvage regimen and consolidate with alloHSCT

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15
Q

What is a bad choice of drug for CLL patients who have autoimmune hemolytic anemia?

A

Fludarabine because purine analogs can also cause AIHA

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16
Q

In what CLL patients is Ofatumumab indicated?

A

(Anti-CD20) it is indicated in patients who have either failed alemtuzumab + fludarabine or in patients who are not candidates for alemtuzumab tx

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17
Q

t(v;11) suggests a mutation in what gene?

A

MLL (Mixed Lineage Leukemia)

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18
Q

What are some signs of differentiation syndrome?

A

Fever, elevation of WBC count, dyspnea with increasing oxygen requirement and pleural + pericardial effusions; CXR often showing diffuse interstitial pulmonary infiltrates

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19
Q

What is the cure for CLL?

A

Allo-HSCT

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20
Q

What are the first line drugs for Hairy Cell Leukemia?

A

Cladribine and Pentostatin (both are antimetabolites)

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21
Q

What drug is a chimeric CD20 inhibitor?

A

Rituximab

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22
Q

If using lenalidomide in CLL, in addtion to hypercoagulability, what is a common AE?

A

Tumor Flare

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23
Q

Explain what the granules of promyelocytes have to do with differentiation syndrome

A

Promyelocytes have granules and when they differentiate w /ATRA based tx the granules stimulate pulmonary edema and lead to fluid retention

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24
Q

Name 2 drugs that have shown benefit in CLL with 17p del

A

Ibrutinib and Venetoclax

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25
Q

What is Passenger Lymphocyte Syndrome?

A

This is when the B-cells from the donor form antibodies against the recipients red cells and cause hemolysis. Tx = supportive as the donor RBCs will eventually engraft

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26
Q

Can you give 7+3 to pregnant patients?

A

Yes, however there is certainly a real risk to the fetus; choice of anthracycline is unclear

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27
Q

If you want to give Ibrutinib + Rituximab to a patient with CLL but note that pt is on plavix what might be a better option?

A

Idelalisib + Rituximab as Ibrutinib causes bleeding issues

28
Q

What are the two types of cells seen in Large Granular Lyphoctye Leukemia?

A

Generally, either CD3+ cytotoxic T cells or CD3- NK cells with CD57 + being the most common of the NK cell variety

29
Q

What can you say about the loss of 17p in CLL?

A

Predictive for lack of benefit from chemotherapeutics and monoclonal ab therapies; tx with Ibrutinib and Venetoclax have shown benefit

30
Q

After what period of time can live vaccines be safely given to BMT patients?

A

2 years, the risk is highest up until 2 years

31
Q

What is the characteristic immunophenotype of Hairy Cell Leukemia?

A

CD19+, CD20+, CD103+, CD11c+, CD25+, and CD123+

32
Q

What are the Stage 0-IV of the Rai staging system for CLL?

A

Stage 0 = ALC >5000 only; Stage 1 = ALC >5,000 + LAD; Stage 2 = ALC >5000 + HSM; Stage 3 = ALC>5000 +anemia (not AIHA); Stage IV = ALC >5000 + thrombocytopenia (not ITP/Evans)

33
Q

What is the mechanism of action of Defibrotide? Where does it come from?

A

It protects the endothelium of the small veins of the liver, used to tx VOD from acute GVHD (6.25 mg/kg IV qid); unknown MOA. Comes from intestinal mucosa of pigs

34
Q

What kind of lymphocytes are NOT affected by Ibrutinib?

A

T lymphcytes; so you do not have same PCP risk as with other CLL drugs

35
Q

What is the driver mutation in Hairy Cell Leukemia?

A

It is almost always BRAF V600E which activates the MEK-ERK pathway (vemurafenib has shown efficacy in some case reports but first line is cladribine)

36
Q

What is FCR?

A

Fludarabine + Cyclophosphamide + Rituximab, used in CLL

37
Q

Ibrutinib is approved for first line tx in CLL pts with _____

A

17p del

38
Q

What drug is a humanized CD20 inhibitor?

A

Ofatumumab

39
Q

What source of HSCs is not ideal in patients with a large BMI?

A

Cord blood due to the low volume; will lead to delayed hematopoeitic recovery

40
Q

What is the caveat about CEBPA mutations in AML and favorable prognosis?

A

There must be biallelic CEBPA mutations, just one is NOT associated with good prognosis

41
Q

What should you think if you have prolonged lymphocytosis on Ibrutinib?

A

It may be due to a quiescent clone but can be due to the ibrutinib itself

42
Q

Mutations in what gene lead to ibrutinib resistance?

A

CXCR4 C481S mutations because Ibrutinib is a BTK inhibitor that binds to C481 of the kinase covalently at a sulfhydryl group resulting in irreversible inhibition of the kinase

43
Q

What is the role of MLL gene in AML pathogenesis? What mutations associated with this? Drug?

A

MLL encodes a histone methyltransferase that maintains gene expression during hematopoeisis, MLL fusion proteins i.e. 11q23 amplifications lead to fusion proteins that directly bind DNA and cause transcription = more cell division; topoisomerase II inhibitors can cause these mutations

44
Q

What conditioning regimen has been shown to increase risk of VOD?

A

Cyclophosphamide + Busulfan as well as Gemtuzumab ozogamicin

45
Q

If the Rai staging system places a patient at either stage III or stage IV what needs to be ruled out?

A

Need to rule out AIHA or ITP (i.e. Evans syndrome) so it needs to be from true BM failure d/t CLL and not autoimmune phenomena

46
Q

A CLL patient with bleeding issues should not receive ______

A

Ibrutinib

47
Q

Ibrutinib is good for upfront tx in what specific cytogenetic risk CLL

A

17p del as this has been shown to predict for lack of responsiveness to traditional upfront chemo

48
Q

What is the MOA of Ibrutinib?

A

It is a BTK inhibitor and causes apopotosis, downregulation of CD20 (target of Rituximab/Ofatumumab), and prevents cells from responding to survival stimuli of the bone marrow microenvironment

49
Q

Which drug is used to treat veno-occlusive disese from acute GVHD?

A

Defibrotide 6.25 mg/kg IV qid. Comes from intestinal mucosa of pigs

50
Q

A person just got an allo-HSCT and is now presenting with jaundice, ascites, and elevated liver enzymes

A

Veno-occlusive disease (VOD) which is injury to the small veins of the liver due to aGVHD

51
Q

What should you do if you have a CLL patient with recurrent sinopulmonary infections?

A

Send for immunoglobulin levels; if hypogammaglobulinemic can then give IVIG

52
Q

In patients with relapsed Hairy Cell Leukemia after cladribine induction what should be tried?

A

If relapse within 1 year can do pentostatin w/ or w/o rituximab and if >1 year can re-challenge with cladribine

53
Q

A seminal study in 2013 showed that this conditioning regimen is best for AML _____ but this myeloablative regimen has less toxicity and similar survival ________

A

Cyclophosphamide + Busulfan; Fludarabine + Busulfan (Flu/Bu/TBI)

54
Q

A mutation in IDH2 in AML is assoc with ______ prognosis

A

Good; IDH1 is not only IDH2

55
Q

These are HLADR-, CD34-, MPO+, and CD33+

A

Acute Promyelocytic leukemia

56
Q

Generally speaking, t(8;21) can be cured with 7+3 and HiDAC consolidation; what molecular mutation causes a worse prognosis in this group?

A

Mutant C-kit

57
Q

What indolent leukemia is often associated with RA, splenomegaly, anemia, and neutropenia and is treated with MTX + Cyclosporine

A

t-LGL (t- large granular lyphocyte leukemia

58
Q

What lymphocyte doubling time is an indication to start tx for CLL?

A

Doubling time of <6 months

59
Q

What is a common AE of fludarabine that can also be caused by CLL itself?

A

AIHA

60
Q

What can you say about deletion of 13q in CLL?

A

This is the most favorable cytogenetic change in CLL

61
Q

When should you discontinue ATRA in the setting of differentiation syndrome?

A

Only if you have started dexamethasone 10 mg IV daily and it still continues

62
Q

How do you manage differentiation syndrome?

A

Continue ATRA but give dexamethasone 10 mg IV daily x3-5 days. Consider stopping ATRA if progresses on steroids

63
Q

Mutant C-kit causes a worse prognosis on what generally good prognostic group of AML?

A

t(8;21) so if you have this can generally do 7+3 followed by HiDAC but if mutant C-kit may need alloHSCT

64
Q

Live vaccines can generally be given 2 years after HSCT but what are some caveats at this point

A

They should be without GVHD and not on ongoing immunosuppression prior to receiving vaccines

65
Q

What is the MOA of Idelalisib?

A

It is an inhibitor of the PI3K (delta isoform) and inhibits the ATP catalyzing domain; causes apoptosis and inhibits signaling through BCR (B cell Receptor) as well as CXCR4 and CXCR5 signaling which affects homing to lymph nodes and BM

66
Q

What is good upfront chemo for CLL patients that are frail or have significant comorbidity and cannot tolerate purine analogs?

A

Obinutuzumab + chlorambucil

MALIGNANT HEME (12 decks)

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