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

WM facts Flashcards

1
Q

Median age WM

A

71

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2
Q

M:F WM

A

3:1

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3
Q

Anti-MAG IgM clinical syndrome

A

Chronic demyelinating neuropathy
Monotherapy with R
50% ORR

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4
Q

MYD88 in WM

A

L265P
93%
not diagnostic (seen in 5% of MZL)
not clear if prognostic
concordant with the extent of BM involvement

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5
Q

CXCR4 mutations in WM

A

30%
but 50% of pts with hyperviscosity
shorter treatment free survival
Ibrutinib resistance
Zanu seems to be effective

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6
Q

Rissk factors for IgM MGUS advancement to WM

A

abnormal kappa/lambda ratio
M protein > 1.5
MYD88 mutation

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7
Q

Symptomatic hyperviscosity in WM %

A

13%

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8
Q

Risk factors in WM to require Tx

A

IgM > 4500 mg%
BM involvement > 70%
B2MG > 4
Alb< 3.5

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9
Q

S/Sx of hyperviscosity syndrome

A

Epistaxis
Gingival bleeding
Retinal hemorrhage
CVA

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10
Q

Tx of hyperviscosity syndrome in WM

A

Plasmapheresis
Usually once is enough
Response for around 2 weeks
Start Tx ASAP

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11
Q

Cutoff of hyperviscosity syndrome

A

Serum viscosity > 4
Usually, there is no hyperviscosity if IgM < 4

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12
Q

Rituximab risk in WM

A

Flare of hyperviscosity

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13
Q

1st line Tx of WM

A

BR
BTKi
R(rituximab)-Vd

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14
Q

Maintenance Tx in WM

A

No benefit

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15
Q

Tx of IgM nephropathy in WM

A

R monotherapy
With R maintenance

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16
Q

Tx of WM associated hemolytic anemia

A

R monotherapy

17
Q

Tx of symptomatic cryoglobulinemia in WM

A

R monotherapy

17
Q

2nd line Tx in WM

A

Zanubrutinib
R (rituximab) Vd
Venetoclax
Repeat BR
ASCT (in very refractory cases)

18
Q

WM IPSS

A

Age> 65
Hb< 11.5
PLT < 100
b2mg > 3
Monoclonal IgM > 7

Not validated in BTKi era

19
Q

Immunophenotype WM

A

sIgM CD19, CD20, CD22, CD138 +
CD23, CD10 -
CD5 +/-

20
Q

Bing Neel syndrome

A

CNS involvement in WM
Tx with BTKi until clinical improvement
Usually continoues Tx after improvement
CSF may still have lymphoma cells

21
Q

Schnitzler syndrome

A

Urticaria associated with WM

22
Q

WM relation to IDA

A

WM secretes hepcidin
causing IDA
not indication to treat WM

23
Q

WM most common cytogenetic abormality

A

Del 6q
IgH translocations are not common

24
Q

IgM MGUS progression to WM

A

2% / year

25
Q

Highest risk mutations for DLBCL in WM pts

A

MYD88wt and CXCR4wt

26
Q

BR advatage over other Tx in WM

A

PFS over R CHOP
Not compared to RVD but PFS seems longer
Not compared to BTKi

27
Q

Zanu vs Ibrutinib in WM

A

Same efficacy
Less SE

28
Q

Pts with WM who acheive PR after BR

A

If ASx do not start 2nd line Tx

Erez's HEM TEST notes 2024 (35 decks)

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