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Erez's HEM TEST notes 2024 > MDS facts > Flashcards

MDS facts Flashcards

1
Q

Incidence of MDS

A

3-4/100K

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2
Q

IPSS-R in MDS

A

Hb < 10, 8
PLT < 100, 50
ANC < 800
Blasts> 2, 5, 10
Cytogentics

Most pts are old so age is not a risk factor

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3
Q

IPSS-M in MDS

A

Hb, PLT, ANC
Blasts
Cytogenetics
Molecular data

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4
Q

Cytogenetic risk in MDS

A

-Y, del 11- very good
Normal, del(5), del(12), del(20)- good
del(7), +8, +19, i(17p)- intermediate
-7, inv(3)/t(3q),del(3q), complex (3 abnormalities)- poor
> 3 abnormalities- very poor

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5
Q

Important mutations in MDS

A

P53
MLL
FLT3
EZH2
ASXL1
TET2
RUNX1
U2AF1
NRAS

SF3B1- favorable

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6
Q

ICUS

A

Cytopenia with no dysplasia and no clonality
Usually resolves spontaneously

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7
Q

CCUS

A

cytopenia with no dysplasia with clonal somatic mutations
carries a higher risk to develop MDS/AML
consider clinical trial

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8
Q

CHIP

A

Clonal mutations without cytopenias
carries a small risk for AML development

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9
Q

Luspatercept vs EPO in LR-MDS

A

COMMANDS trial
Luspa has better transfusion independence rates (60% vs 30%)
Mainly in SF3B1/ RS

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10
Q

Lenalidomide effectiveness in low risk MDS

A

In del(5q)
70% transfusion independence

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11
Q

Timing of Len Tx in low risk MDS

A

Possibly before transfusion dependence
May reduce the need for future transfusions and maybe OS

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12
Q

Tx with HMAs in low risk MDS

A

Use low doses and short (5 days a month) courses
Mainly when multilnge dysplasia
Usually, after luspa or EPO failure
Not approved in EMA

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13
Q

Imetelstat in low risk MDS

A

Telomerase inhibitor
iMERGE trail
Phase III
vs placebo
Effective in pts who failed EPO
~40% TI

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14
Q

Oral decitabine-cedazuridine vs IV decitabine in high risk MDS

A

ASCERTAIN trial
Phase III
Included CMML
Also in p53
Same PK and PD
60% ORR 21% CR

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15
Q

AZA Tx for high risk MDS

A

AZA-001
compared to BSC
OS advantage!!!
24 months vs 15 months

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16
Q

AZA +- Ven for high risk MDS

A

VERONA trial
no results yet

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17
Q

VEXAS

A

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic
UBA1 gene
Men over 50
Fever, cytopenias, inflammation, thrombosis
Tx is with steroids, JAK2i, HMA, and transplant

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18
Q

Dysplasia threshold in MDS

A

10%

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19
Q

Copper deficiency presentation

A

Post bariatric surgery
MDS +
Myelopathy/neuropathy

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20
Q

MDS with del5q clinical menifistations

A

Anemia
Thrombocytosis!
Megakaryocytes with monolobated nuclei

Defined as del5 with only one other cytogenetic abnormality, except monosomy 7 or del(7q).

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21
Q

Significance of LGL/PNH clones in MDS

A

Better response to immunosuppressive therapy

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22
Q

LR-PSS in MDS

A

A prognostic model to find poor risk pts with low risk MDS
Age, TD, cytogenetics found to be important

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23
Q

IPSS-R in MDS cutofff

A

3.5
Above is considered high risk

24
Q

Factors related to response to TPO-RAs in MDS

A

TPO<500
Low risk
Limited PLT transfusions

25
Q

TPO-RAs in high risk MDS

A

ASPIRE trial
Less thrombocytopenia but same rate of bleeding
May induce proliferation of blasts in high risk pts

26
Q

Deferasirox effectiveness in low risk MDS

A

Improves EFS and composite outcome that included OS

27
Q

When to start iron chelation in low risk MDS

A

After 20-30 PC
Usually ferritin > 1000

28
Q

Deferasirox precautions

A

PO

Hepatic/renal impairment
GI bleeding
Decreased hearing

29
Q

Luspatercept effectiveness in low risk MDS

A

COMMANDS trial
Phase III
vs EPO
Primary end point of TI
Effective in SF3B1 mutated and maybe also in all low risk MDS

30
Q

Non HMA option for LR MDS pts with MLD

A

ATG+prednisone
50% ORR

31
Q

% with ctogenetic abnormalities in MDS

A

50%

32
Q

Risk factors for progression of CCUS to AML

A

High VAF
More than 1 mutation
IDH1
p53

33
Q

Median age MDS

A

76
>90% above 50

34
Q

% with genetic mutations in MDS

A

90%

35
Q

MDS with fibrosis

A

BM blasts 5-19%

36
Q

Erythroid MDS changes

A

Ring sidroblasts>15%
Megaloblastic changes
Multinuclear
Cytoplasmmic fraying

37
Q

TET2 in MDS

A

Good prognosis

38
Q

Tx of HR MDS that progressed on vidaza

A

Add venetoclax

39
Q

Response to AZA in p53 MDS

A

Also respond

40
Q

Tx of CCUS

A

Clinical trial
Vitamin C may be effective

41
Q

Worst mutations in CCUS

A

p53
IDH2
almost 100% will develop AML

42
Q

GATA2 deficiency syndrome

A

MDS/AML at age 20
Autism
ADHD
Hearing loss
Thrombosis

43
Q

Prevalence of germline DDX41

A

1.5% of MDS/AML
Most common familial disorder in adults
Older age

44
Q

Diagnosis of Fanconi anemia

A

Chromosome breakage in PB or skin

45
Q

Lenalidomide in 5q MDS

A

10 mg
Early cytopenias are positive predictive markers for response
Rapid and sustained responses

46
Q

EPO SE

A

HTN
Headache
VTE
Arthralgia
Rash
N/V

47
Q

Differences between IPSS and IPSS-R

A

IPSS-R puts more significance on cytogenetic changes and has a different blast percentage cutoff

48
Q

MDS defining abnormalities (dysplasia not mandatory)

A

SF3B1
del5q
>5% blasts

Usually dysplasia is present but it is not mandatory

49
Q

Fanconi anemia
diagnosis

A

Chromosome breakage
Preferably from fibroblast
But usually from PB

50
Q

Benefit of chelation in MDS

A

Maybe OS
But non randomized studies
And composite outcome of moratilty and iron overload

51
Q

IDUS

A

Idiopathic dysplasia unknown significance
Dysplasia without cytopenias or clonality

52
Q

IDUS

A

Idiopathic dysplasia unknown significance
Dysplasia without cytopenias or clonality

53
Q

MDS classification WHO

A

MDS with defining genetic abnormalities (5q, SF3B1, p53)
MDS morphological defined (low, IB1, IB2, hypoplastic, fibrosis)

54
Q

MDS classification ICC

A

5q, SF3B1, p53
Single dysplasia, multilineage dysplasia, no dysplasia, EB, MDS/AML

55
Q

Common CHIP mutation in smokers

A

ASXL1

Erez's HEM TEST notes 2024 (35 decks)

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