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Hematology, oncology > Myelodysplastic syndrome > Flashcards

Myelodysplastic syndrome Flashcards

1
Q

Lab profile (features of anemia and other thrombocytopenias)

A
  • Anemia: low retic count + commonly macrocytic
  • leukopenia (50%)
  • varying degrees of thrombocytopenia (25%)
  • dysplastic cells on smear (≥10 percent of erythroid precursors, granulocytes, or megakaryocytes)
  • inappropriately low retic count
  • blasts <20%
  • commonly monocytosis
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2
Q

Indications for treatment of lower risk MDS

A

1) Requiring frequent transfusions

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3
Q

Initial therapy for intermediate-2/high-risk MDS

A

IF non candidate for HSCT → azacitidine or until disease progression or intolerable side effects
IF candidate → AlloHSCT

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4
Q

pathophysiology of MDS

A

MDS is thought to arise from mutations in the *multipotent stem cell, but the specific defects responsible for these diseases remain poorly understood. Differentiation of blood precursor cells is impaired, (this leads to multiple cytopenias) and a *increase in apoptotic cell death occurs in bone marrow cells.

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5
Q

venetoclax mechanism

A

Blocks the anti-apoptotic B-cell lymphoma-2 (Bcl-2) protein, leading to programmed cell death of CLL cells.

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6
Q

Management of patient with clinically significant anemia

A

1) measure epo. if below 500, give epo.

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7
Q

What are the hypomethylating agents?

A

azacetadine or decitabine

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8
Q

Risk stratification score used for MDS

A

Revised International Prognostic Scoring System (IPSS-R)

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9
Q

General diagnosis of MDS

A

1) Cytopenias
2) Low blast percentage in marrow
3) Morphologic evidence of dysplasia

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10
Q

Workup of suspected MDS

A
  • **peripheral smear

- bone marrow aspirate and biopsy

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11
Q

MDS-SLD means

A

MDS-single lineage dysplasia (1 or 2 cytopenias, but other cell lines are normal)

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12
Q

What are the dysplastic cells you may see on peripheral smear indicating MDS?

A

≥10 percent of erythroid precursors, granulocytes, or megakaryocytes

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13
Q

Diagnostic criteria

A

1) cytopenia in at least one blood lineage (but can have single lineage dysplasia MDS)
2) dysplasia in 10% or more of nucleated cells in at least one lineage
3) less than 20% blasts in blood and bone marrow
4) and/or characteristic cytogenetic findings

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14
Q

what does CHIP stand for?

A
  • clonal hematopoiesis of indeterminate potential
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15
Q

what does risk stratification depend on?

A
  • percentage of bone marrow blasts
  • number and severity of cytopenias
  • cytogenetic findings
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16
Q

Examples of lower-intensity treatments

A
  • epoetin alfa
  • thrombopoietin receptor agonists
  • neupogen
  • luspatercept
  • hypomethylating agents (HMA; eg, azacitidine, decitabine)
  • immunosuppressive therapy, lenalidomide
  • targeted agents (eg, IDH inhibitors).
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17
Q

Actionable mutation in MDS

A

IDH1 or IDH2

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18
Q

Adverse genetic features in MDS

A

1) TP53 mutation

2) adverse cytogenetic abnormalities

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19
Q

Management of high risk MDS patient

A

IF adverse genetic features present, HSCT is preferable
IF not, then targeted therapy with IDH1 agent is preferable
IF no IDH1, then transplant

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20
Q

what are the IDH inhibitor drugs?

A

enasidenib and ivosidenib

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21
Q

procrit generic name

A

EPO

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22
Q

Marrow in MDS

A
  • typically hypercellular but can be hypocellular (hypocellular MDS)
23
Q

how to determine expected cellularity of marrow in a patient

A

cellularity = 100 - age

24
Q

Most favorable mutation

A

SF3B1

25
Q

FDA approved drug for MDS w/ ring sideroblasts

A

luspatercept

26
Q

Combination therapy approved for MDS

A

decitabine and cedazuridine (inqovi)

27
Q

WHO categories for MDS

A 
MDS with single lineage dysplasia
MDS with multilineage dysplasia
MDS with ring sideroblasts
MDS with excess blasts
MDS with isolated del(5q)
28
Q

NCCN preferred/category 1 HMA for higher risk

A

azacitadine

29
Q

Role for IDH inhibitors

A

Ongoing research, second line (confirm, not sure)

30
Q

Dysplastic features on smear or marrow aspirate suggesting MDS

A

Ovalomacrocytosis is the most common morphologic abnormality, dysplastic PMNs, elliptocytes, teardrops, stomatocytes, or acanthocytes (spur cells), basophilic stippling, Howell-Jolly bodies, hypolobulated megakaryocytes megaloblastoid nucleated red cells

31
Q

When is lenalidomide used in MDS

A

5q deletion, low and intermediate risk MDS, with anemia

32
Q

what are the IPSS-R risk categories?

A
  • low
  • intermediate-1
  • intermediate-2
  • high
  • very high
33
Q

Staging system for MDS

A

IPSS-R

34
Q

Number of blasts required for MDS with excess blasts-1

A

5-9%

35
Q

Number of blasts required for MDS with excess blasts-2

A

10-19%

36
Q

Auer rods in MDS?

A

You can have auer rods in MDS-EB2

*Not all auer rods are APML

37
Q

frequency of chromosomal translocations in MDS vs. AML

A

less common in MDS

38
Q

Management of patient with characteristics AML cytogenetic abnormalities (8;21, 15;17, inversion 16)

A

3+7 chemotherapy

39
Q

Indication for lenalidomide

A

Transfusion-dependent patients with low or intermediate-1 MDS + 5q deletion

40
Q

duration of azacitadine in MDS

A

Continue until progression or unacceptable toxicity (data shows interruption of HMAs is associated with earlier relapse and poor prognosis)

41
Q

why azacitidine vs. decitabine

A
  • haven’t been compared head to head

- only aza has demonstrated a survival benefit, so it is in guidelines

42
Q

azacitidine and decitabine formulation

A
  • aza = can also be PO or IM

- decitabine is IV

43
Q

Variables included in IPSS-R

A

1) cytogenetics
2) blasts
3) hgb
4) plt
5) ANC

44
Q

trade name for azacitadine

A

vidaza

45
Q

what is inqovi

A

Oral combination of decitabine + cedazuridine

46
Q

Preferred HMA for non-transplant candidates

A

azacitidine

47
Q

generic name for aranesp

A

darbopoeitin

48
Q

Treatment durations for HMAs with MDS

A
  • Until progression or unacceptable toxicity (data shows that interruption of HMAs in a patient who is responding is associated with an earlier relapse and a poor prognosis)
49
Q

Can MDS be cured?

A

Allogeneic hematopoietic stem cell transplantation is the only curative therapy for MDS

50
Q

Worst mutation to have in MDS

A

TP53

51
Q

What is CCUS?

A

clonal mutation + cytopenia BUT doesn’t meet WHO criteria for a heme neoplasm

52
Q

Target hgb range in MDS

A

10

53
Q

Very good cytogenetic findings in MDS

A

Deletion Y, del 11

Hematology, oncology (218 decks)

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