WM facts Flashcards
Median age WM
71
M:F WM
3:1
Anti-MAG IgM clinical syndrome
Chronic demyelinating neuropathy
Monotherapy with R
50% ORR
MYD88 in WM
L265P
93%
not diagnostic (seen in 5% of MZL)
not clear if prognostic
concordant with the extent of BM involvement
CXCR4 mutations in WM
30%
but 50% of pts with hyperviscosity
shorter treatment free survival
Ibrutinib resistance
Zanu seems to be effective
Rissk factors for IgM MGUS advancement to WM
abnormal kappa/lambda ratio
M protein > 1.5
MYD88 mutation
Symptomatic hyperviscosity in WM %
13%
Risk factors in WM to require Tx
IgM > 4500 mg%
BM involvement > 70%
B2MG > 4
Alb< 3.5
S/Sx of hyperviscosity syndrome
Epistaxis
Gingival bleeding
Retinal hemorrhage
CVA
Tx of hyperviscosity syndrome in WM
Plasmapheresis
Usually once is enough
Response for around 2 weeks
Start Tx ASAP
Cutoff of hyperviscosity syndrome
Serum viscosity > 4
Usually, there is no hyperviscosity if IgM < 4
Rituximab risk in WM
Flare of hyperviscosity
1st line Tx of WM
BR
BTKi
R(rituximab)-Vd
Maintenance Tx in WM
No benefit
Tx of IgM nephropathy in WM
R monotherapy
With R maintenance
Tx of WM associated hemolytic anemia
R monotherapy
Tx of symptomatic cryoglobulinemia in WM
R monotherapy
2nd line Tx in WM
Zanubrutinib
R (rituximab) Vd
Venetoclax
Repeat BR
ASCT (in very refractory cases)
WM IPSS
Age> 65
Hb< 11.5
PLT < 100
b2mg > 3
Monoclonal IgM > 7
Not validated in BTKi era
Immunophenotype WM
sIgM CD19, CD20, CD22, CD138 +
CD23, CD10 -
CD5 +/-
Bing Neel syndrome
CNS involvement in WM
Tx with BTKi until clinical improvement
Usually continoues Tx after improvement
CSF may still have lymphoma cells
Schnitzler syndrome
Urticaria associated with WM
WM relation to IDA
WM secretes hepcidin
causing IDA
not indication to treat WM
WM most common cytogenetic abormality
Del 6q
IgH translocations are not common
