Waldenstrom Macrolgobulinemia

Question 1

Waldenstrom’s basic pathophysiology

Answer 1

• malignancy of mature, plasmacytoid lymphocytes that secrete IgM
• (lymphoplasmacytic lymphoma)
• it is considered to be a low-grade B-cell lymphoma

Question 2

presentation

Answer 2

Patient denies drenching night sweats, fever, weight loss, and anemia. Patient denies symptoms of hyperviscosity (altered vision, headache, hearing loss, tinnitus, dizziness, nystagmus, altered mental status, and nasal and oropharyngeal bleeding). Patient denies neuropathy.

Question 3

disease associated with waldenstrom’s

Answer 3

hep C

Question 4

indications for treatment

Answer 4

hyperviscosity, organomegaly, cryoglobulinemia, cold agglutinin disease, cytopenia

Question 5

treatment of hyperviscosity in waldenstrom’s

Answer 5

plasmapheresis

Question 6

How to differentiate WM from IgM

Answer 6

1) MYD88 L265P gene mutation (present in over 90%) of patients with WM)
2) BMB with characteristic immunophenotype of WM

Question 7

complications

Answer 7

• cold agglutinin hemolytic anemia
• amyloidosis
• neuropathy
• cryoglobulinemia
• renal failure
• organomegaly

Question 8

hyperviscosity syndrome management

Answer 8

plasmapheresis

Question 9

serum viscosity that indicates patient is at high risk of developing hyperviscosity

Answer 9

greater than 4

Question 10

Indications for plasmapheresis before induction therapy

Answer 10

1) ***rituxan containing regimen and IgM >4K

2) symptomatic hyperviscosity

Question 11

Indications for starting treatment

Answer 11

hyperviscosity, hepatosplenomegaly, cryoglobulinemia, cold agglutinin disease, b symptoms
bulky adenopathy
disease-related hgb <10
platelets <100k
neuropathy
amyloidosis

Question 12

Induction regimen for WM patients presenting with symptomatic hyperviscosity

Answer 12

bortezomib/dexamethasone/rituxan

Question 13

Diagnosis

Answer 13

monoclonal IgM protein + 10% or greater clonal LPL cells in the bone marrow

Question 14

most common mutation

Answer 14

L265P mutation in MYD88 gene

Question 15

characteristic clinical finding in WM

Answer 15

retinopathy

Question 16

First line options for WM

Answer 16

BR
Rituxan/cytoxan/dex
bortezomib/dex/rituxan
zanubrutinib
ibrutinib +/- rituxan

Question 17

second line

Answer 17

Ibrutinib +/- rituxan

zanubritinib

Question 18

HIV increases risk for what cancer types?

Answer 18

HL and NHL (even despite HAART)

Question 19

Serum viscosity at which patients are at high risk of developing hyperviscosity syndrome

Answer 19

Greater than 4

Question 20

Drug that can cause hyperviscosity symptoms to flare with WM

Answer 20

Rituximab

Question 21

Characteristic marrow finding with Waldenstrom’s

Answer 21

Lymphoplasmacytic lymphoma cells

Question 22

management of smoldering waldenstrom’s

Answer 22

observation