WM, Amyloidosis, and other PC Dyscrasias

Question 1

Most frequently seen cytogenetic abnormality in WM?

Answer 1

6q del

Question 2

Immunophenotype for WM

Answer 2

sIgM+
CD5+/-, Cd10-, CD19+, CD20+, CD22+, CD23-, CD138+

Question 3

Most common mutation in WM?

Answer 3

MYD88 (80-100% of patients)

Question 4

What mutation is associated with WHIM syndrome?

Answer 4

CXCR4

Question 5

What are 4 risk factors for progression from asymptomatic WM to symptomatic?

Answer 5

BM >70%
IgM >4500
B2m >4
Albumin <3.5

Question 6

What is Bing Neel syndrome/

Answer 6

WM with CNS involvement

Question 7

What are the 5 parts of the WM IPSS? How many RFs do you need to be high risk?

Answer 7

Age >65
Hgb <11.5
Platelet <100
B2m >3
M protein >7 g/dL

high risk >2

Question 8

What specific toxicity can occur with Rituximab therapy in WM?

Answer 8

IgM flare (within first 4 months) which can cause hyperviscosity, worsening WM related peripheral neuropathy, CAD
or hypogammaglobulinemia

Question 9

1L treatment for WM with low IgM and mild symptoms

Answer 9

Rituximab

Question 10

1L treatment for WM with mod/high IgM or significant symptoms (2)

Answer 10

BR
CyD + R

Question 11

Treatment with WM with MAG neuropathy (2)

Answer 11

Ritux
Ibrutinib

Question 12

Teratment for Bing Neel syndrome (2)

Answer 12

Ibrutinib
Zanu

Question 13

2L treatment of WM

Answer 13

BTKi

Question 14

Preferred induction treatment for AL amyloidosis

Answer 14

Dara-CyBorD

Question 15

2L treatment of AL amyloid with no prior daratumumoab or PI

Answer 15

Dara-VD
HSCT if a candidate

Question 16

3L treatment of AL amyloidosis, those who are dara/PI refractory

Answer 16

IMID or
alkylating agent

Question 17

Treatment of RR AL amyloidosis with t(11;14)

Answer 17

Venetoclax

Question 18

Diagnostic criteria for POEMS

Answer 18

2 mandatory, 1 major and 1 minor
Mandatory: Demyelinating polyneuropathy, monoclonal PC disorder
Major: Sclerotic bone lesion, elevated VEGF, Castleman disease
Minor: Volume overload, organomegaly, endocrinopathy, skin changes, papilledema, polycythemia and thrombocytosis

Question 19

Treatment of POEMS with 1-3 sclerotic lesinos with no marrow involvment

Answer 19

RT 40-50 Gy

Question 20

Treatment of POEMS with marrow involvement

Answer 20

RD
ASCT if eligible

Question 21

What protein accumulates and deposits into tissues in secondary amyloid?

Answer 21

Amyloid A
It’s an acute phase reactant

Question 22

Treatment for neuropathy caused by hereditary TTR amyloid? (2)

Answer 22

Patisiran
Inotersen
Eplontersen

Question 23

Treatment for ATTR cardiomyopathy?

Answer 23

Tafamidis