ALEXANDER(GRUNENBERG((ULM)(
(
MARGINAL(ZONE(LYMPHOMA(?(CASE(REPORTS(
Background:!(
Marginal!zone!lymphoma!(MZL)!represents!a!group!of!indolent!B>cell!lymphomas.!According!
to! the! WHO! classification! of! non>Hodgkin! lymphoma,! MZL! comprises! three! main! entities:!
splenic! (SMZL),! nodal! (NMZL)! and! extranodal! MZL! (EMZL)! of! mucosa>associated! lymphoid!
tissue!type!of!which!the!latter!represents!by!far!the!most!common!subgroup.(
!
EMZL! is! the! most! common! entity! of! all! MZL.! Infiltration! occurs! in! a! variety! of! extranodal!
organs,! usually! in! the! context! of! chronic! antigenic! stimulation! due! either! to! infections! or!
autoimmune!disorders!with!the!stomach!as!the!most!affected!site.!
!
The! diagnosis! of! NMZL! is! made! only! after! excluding! other! indolent! lymphomas! and! is!
characterized! by! enlarged! lymph! nodes,! which! can! be! localized! or! widespread.! As! the!
neoplastic!counterpart!postgerminal!center!marginal!zone!cells!are!discussed.!Histologically,!
NMZL!are!sometimes!difficult!to!distinguish!from!other!indolent!lymphomas!and!diagnosis!
requires!the!exclusion!of!extranodal!manifestations!and!splenic!involvement.!
!
SMZL! is! frequently! diagnosed! as! an! incidental! finding! of! lymphocytosis,! whereas! marked!
splenomegaly!as!well!as!anemia!and!thrombocytopenia!are!prominent!features!in!advanced>
stage! disease.! The! prognosis! of! SMZL! is! heterogenous,! including! a! third! of! patients! with! a!
worse!prognosis.!Definitive!diagnosis!of!SML!is!based!on!splenectomy!but!in!practice!is!often!
replaced! by! combining! morphological! and! immunophenotypical! diagnostics! of! peripheral!
blood!and!bone!marrow!cells.!
!
The!2016!update!of!the!WHO!classification!of!lymphoid!neoplasms!acknowledges!the!term!
monoclonal! lymphocytosis! of! marginal! zone! origin.! There! are! only! a! few! data! about! this!
entity.! This! condition! is! rather! indolent! and! rarely! progresses! to! a! well>recognized!
lymphoma.!
!
Basically,!there!are!three!treatment!options!for!MZL!lymphoma.!These!include!anti>infective!
therapy,! local! therapy! (surgery,! radiotherapy)! and! systemic! therapy,! which! are! shown! in!
Figure!1.!
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