ELIAS(CAMPO((BARCELONA)(
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DIAGNOSTICS(
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!
Mantle! cell! lymphoma! (MCL)! is! a! well>defined! entity! characterized! by! a! proliferation! of!
mature!B!lymphocytes!coexpressing!CD5!and!carrying!the!t(11;14)!translocation!leading!to!
the!constitutive!overexpression!of!Cyclin!D1.!The!diagnosis!is!relatively!easy!when!the!tumor!
has! the! classical! morphological! and! phenotypic! features.! However,! MCL! may! have! a!
spectrum!of!cytological!and!phenotypic!variants!that!may!make!the!diagnosis!challenging.!In!
fact,!in!some!reproducibility!studies!between!general!and!referral!hospitals!the!diagnosis!of!
MCL!may!be!missed!in!more!than!25%!of!the!cases!(1).!The!small!cell!variant!of!MCL!may!be!
confused!with!chronic!lymphocytic!leukemia!whereas!blastoid!and!pleomorphic!variants!may!
rise! the! differential! diagnosis! with! lymphoblastic! and! diffuse! large! B>cell! lymphoma!
respectively.!One!variant!that!may!be!difficult!to!diagnose!is!MCL!cyclin!D1!negative.!These!
cases! have! similar! pathological! features! as! cyclin! D1! positive! cases! but! do! not! carry! the!
t(11;14)!translocation.!Cyclin!D1!negative!MCL!express!the!transcription!factor!SOX11!as!the!
conventional!cyclin!D1>!positive!cases!and!therefore!this!marker!is!very!useful!to!recognize!
these!cases.!!Genetic!and!recent!whole!genomes!studies!have!determined!that!these!cases!
carry!translocations!of!CCND2!and!CCND3!with!IG!genes,!referentially!light!chain!and!in!some!
cases!cryptic!insertions!of!the!enhancer!of!the!light!chain!genes!in!the!vicinity!of!these!genes!
leading!to!the!overexpression!of!cyclin!D2!and!D3,!respectively.(2)!Some!cases!of!MCL!have!a!
leukemic! presentation! without! apparent! lymphadenopathy.! Some! of! these! patients! may!
develop! splenomegaly! without! lymph! nodes.! The! clinical! course! of! these! patients! seems!
more!indolent!than!cases!presenting!with!lymphadenopathy.!This!variant!has!been!named!
leukemic! non>nodal! and! the! tumor! cells! may! express! CD23! and! CD200! that! are! usually!
negative! in! conventional! MCL.! These! cases! are! also! negative! for! SOX11! whereas!
conventional! MCL! is! positive.(3)! Recent! genomic! studies! have! defined! the! different!
mutational! and! genomic! profiles! of! these! two! clinic>pathological! variants! of! MCL.! In! both!
variants! the! presence! of! TP53! alterations! and! increasing! genomic! alterations! confer! an!
adverse!prognosis.!(4)!!!!!
(R
eferences(:((
1. Laurent! C! et! al! Impact! of! Expert! Pathologic! Review! of! Lymphoma! Diagnosis:! Study! of! Patients!
From!the!French!Lymphopath!Network.!J!Clin!Oncol.!2017;35(18):2008>2017.!
!!!
2. Martin>Garcia!D!et!al.!CCND2!and!CCND3!hijack!immunoglobulin!light>chain!enhancers!in!cyclin!
D1(>)!mantle!cell!lymphoma.!Blood!2019;133(9):940>951.!
!
3. Puente! XS,! Jares! P,! Campo! E.! Chronic! lymphocytic! leukemia! and! mantle! cell! lymphoma:!
crossroads!of!genetic!and!microenvironment!interactions.!Blood.!2018;131(21):2283>2296.!
!
4. Nadeu!F.!Martin>Garcia!D!!et!al.!Genomic!and!epigenomic!insights!into!the!origin,!pathogenesis,!
and!clinical!behavior!of!mantle!cell!lymphoma!subtypes.!Blood.!2020;136(12):1419>1432.!
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