Waldenstrom Macroglobulinemia

Question 1

First line for WM

Answer 1

• observe unless indication for treatment
• IF indicated for treatment, BR

Question 2

Second line for WM

Answer 2

IF not received firstline, BTK inhibitor
zanubritinib
IF long PFS from frontline, repeat BR

Question 3

Other mutation to know for WM + clinical relevance

Answer 3

CXCR4
- ibrutinib resistance

Question 4

WM management

Answer 4

• if asymptomatic, observe
• if indication for treatment, BR

Question 5

What is Bing Neel syndrome?

Answer 5

WM w/ CNS involvement

Question 6

What is MAG neuropathy

Answer 6

peripheral neuropathy + IgM paraprotein

Question 7

SE’s of rituximab in WM

Answer 7

• worsening hyperviscosity
• worsening IgM related peripheral neuropahty
• worsening cold aggluttinin disease or cryo
• hypogammaglobulinemia

Question 8

Other SE to know for ibrutinib

Answer 8

Myelosuppression (neutropenia, anemia, thrombocytopenia)

Question 9

Second line for WM

Answer 9

BTK inhibitor

Question 10

Amyloid types implicated in hereditary amyloidosis

Answer 10

• Transthyretin (TTR)
• Alpha chain of fibrinogen A

Question 11

ESRD and transplant in amyloidosis

Answer 11

*Do need CrCL >30 in amyloidosis to be eligible for transplant

Question 12

Other POEMS major diagnostic criteria

Answer 12

• sclerotic bone lesions
• elevated VEGF
• Castleman disease

Question 13

Biomarker for POEMS

Answer 13

VEGF

Question 14

POEMS treatment

Answer 14

IF limited disease burden w/ up to 3 sclerotic bone lesions, radiation
IF systemic, len/dex and transplant if eligible

Question 15

PV risk stratification

Answer 15

high = >60 yo OR VTE history