JORGE(J.(CASTILLO((BOSTON)(
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WALDENSTRÖM(MACROGLOBULINEMIA(
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Waldenström! macroglobulinemia! (WM)! is! a! rare! B>cell! lymphoma! characterized! by! the!
accumulation!of!malignant!lymphoplasmacytic!cells!in!the!bone!marrow,!and!other!organs,!
and!a!serum!monoclonal!IgM!paraproteinemia.!Recurrent!somatic!mutations!in!the!MYD88!
and!CXCR4!genes!can!be!detected!in!more!than!90%!and!between!30>40%!of!WM!patients,!
respectively.! Clinically,! WM! can! present! with! anemia,! neuropathy,! hyperviscosity! and/or!
extramedullary! disease! such! as! lymphadenopathy! and! splenomegaly,! and! rarely! can! cause!
pleural! effusions,! renal! dysfunction! and! central! nervous! system! involvement.! However,! as!
WM!is!incurable,!and!WM!patients!have!a!prolonged!overall!survival!measured!in!decades,!
treatment!is!not!indicated!in!asymptomatic!patients.!Bone!marrow!involvement!and!serum!
albumin,! IgM! and! beta>2>microglobulin! levels! can! predict! time! to! treatment! initiation.! The!
treatment! options! for! WM! patients! are! varied! and! include! alkylating! agents,! proteasome!
inhibitors! and! anti>CD20! monoclonal! antibodies.! The! armamentarium! against! WM! has!
expanded! with! the! advent! of! covalent! BTK! inhibitors.! BCL2! inhibitors,! non>covalent! BTK!
inhibitors!and!CXCR4!targeting!agents!are!undergoing!clinical!development!in!WM!patients.!
The!choice!of!therapy!for!WM!patients!should!be!highly!personalized,!having!into!account!
the!clinical,!pathological!and!genomic!features!of!the!disease!as!well!as!patient’s!preference!
and!co>morbidities.!
!
References:(
1. Kyle!RA,!Treon!SP,!Alexanian!R,!Barlogie!B,!Bjorkholm!M,!Dhodapkar!M,!et!al.!Prognostic!markers!
and! criteria! to! initiate! therapy! in! Waldenstrom's! macroglobulinemia:! consensus! panel!
recommendations! from! the! Second! International! Workshop! on! Waldenstrom's!
macroglobulinemia.!Semin!Oncol!2003;!30:!116>120.!
2. Owen! RG,! Treon! SP,! Al>Katib! A,! Fonseca! R,! Greipp! PR,! McMaster! ML,! et! al.! Clinicopathological!
definition! of! Waldenstrom's! macroglobulinemia:! consensus! panel! recommendations! from! the!
Second! International! Workshop! on! Waldenstrom's! Macroglobulinemia.! Semin! Oncol! 2003;! 30:!
110>115.!
3. Castillo! JJ,! Garcia>Sanz! R,! Hatjiharissi! E,! et! al.! Recommendations! for! the! diagnosis! and! initial!
evaluation! of! patients! with! Waldenstrom! macroglobulinaemia:! a! task! force! from! the! 8th!
International!Workshop!on!Waldenstrom!Macroglobulinaemia.!Br!J!Haematol!2016;!175:!77>86.!
4. Treon! SP,! Xu! L,! Yang! G,! Zhou! Y,! Liu! X,! Cao! Y,! et! al.! MYD88! L265P! somatic! mutation! in!
Waldenstrom's!macroglobulinemia.!N!Engl!J!Med!2012;!367:!826>833.!
5. Hunter! ZR,! Xu! L,! Yang! G,! Zhou! Y,! Liu! X,! Cao! Y,! et! al.! The! genomic! landscape! of! Waldenstrom!
macroglobulinemia!is!characterized!by!highly!recurring!MYD88!and!WHIM>like!CXCR4!mutations,!
and! small! somatic! deletions! associated! with! B>cell! lymphomagenesis.! Blood! 2014;! 123:! 1637>
1646.!
6. Castillo! JJ,! Gustine! JN,! Meid! K,! Dubeau! TE,! Severns! P,! Xu! L,! Yang! G,! Hunter! ZR,! Treon! SP.!
Response!and!survival!for!primary!therapy!combination!regimens!and!maintenance!rituximab!in!
Waldenström!macroglobulinaemia.!Br!J!Haematol!2018;!181:!77>85.!
7. Treon!SP,!Tripsas!CK,!Meid!K,!Warren!D,!Varma!G,!Green!R,!et!al.!Ibrutinib!in!previously!treated!
Waldenstrom's!macroglobulinemia.!N!Engl!J!Med!2015;!372:!1430>1440.!
SCIENTIFIC PROGRAMME
SESSION I
HODGKIN’S DISEASE
DEBATE I
IS THERE STILL A ROLE
FOR COMBINED MODALITY
THERAPY FOR EARLY
STAGE CHL?
SESSION II
T-CELL LYMPHOMA
ROUNDTABLE I
FUTURE DIRECTIONS IN
T-CELL LYMPHOMA
SESSION III
FOLLICULAR LYMPHOMA
DEBATE II
CAN WE AVOID
CHEMOTHERAPY IN
THE MANAGEMENT OF
FOLLICULAR LYMPHOMA?
SESSION IV
RARE LYMPHOMAS –
MARGINAL ZONE
LYMPHOMA AND
WALDENSTRÖM M
ACROGLOBULINEMIA
ROUNDTABLE II – WHERE
TO GO IN RARE B-CELL
LYMPHOMAS
SESSION V
MANTLE CELL LYMPHOMA
SESSION VI
DIFFUSE LARGE B-CELL
LYMPHOMA
DEBATE III
DO WE STILL NEED ASCT
IN MCL?
SESSION VII
NOVEL THER APEUTIC
CONCEPTS IN B-CELL
LYMPHOMAS
SELECTED ABSTRACTS
FOR AN ORAL
PRESENTATION
SELECTED ABSTRACTS
FOR A POSTER
PRESENTATION
DISCLOSURES