Waldensrtom's macroglobulinemia

Question 1

BM threshhold of LPLs

Answer 1

No threshhold
But pts with < 10% usually have a indolent course

Question 2

IgM levels for diagnosis

Answer 2

any anount

Question 3

MYD88 %

Answer 3

80-95%

Question 4

Mean age

Answer 4

69

Question 5

Male %

Answer 5

60%

Question 6

Clinical presntation

Answer 6

Anemia 42%
B-symp 25%
Hyperviscosity 17%
Neuropathy 12%
Amyloidosis, Cryo and cold agglutinin are rare

Question 7

Neuropaty

Answer 7

Sensory
Symmetric
Ascending
demyelintaing
Slowly progressing

Question 8

Asymmptomatic pts %

Answer 8

20-30%

Question 9

Score for perdiction of time to therapy ina asymmptomatoc pts

Answer 9

IgM levels
b2mg
Albumin

Question 10

Score for perdiction of time to therapy ina asymmptomatoc pts

Answer 10

BM infiltration
IgM levels
b2mg
Albumin

Question 11

Cytogentics

Answer 11

del6q - 30-50%
No prognostic effect

Question 12

IPSSWM

Answer 12

Age
b2mg
HB
PLT
IgM level

Question 13

rIPSSWM

Answer 13

Age
b2mg
LDH

Question 14

Indications for treament

Answer 14

Symptoms
HB< 10g%
PLT < 100K
Hemolysis
Nephropathy
Neuropathy
Bulky lymphadenopathy (>5 cm)

Question 15

iNNOVATE trial

Answer 15

Ibrutinib + R vs. R vs. placebo
ND
Ibrutinib resolved in 80% reduction in progressoion

Question 16

Choice of 1st line

Answer 16

If tumor burden is high (lymphadenopathy/BM infiltration/B symptoms)- BR or IR are prefered.
If cytopenias predominant- R^2 or IR

Question 17

Indications for Plasmaphersis

Answer 17

Hyperviscosity
Cold agglutinin
Cryoglobulinemia

Question 18

Indications for Plasmapheresis

Answer 18

Hyperviscosity
Cold agglutinin
Cryoglobulinemia