POSTER'43'
MANTLE'CELL'LYMPHOMA'OF'MUCOSAIASSOCIATED'TISSUE:'AN'
ILLUSTRATIVE'CLINICAL'CASE'
'
Ana!Silva!Martins1,!Daniela!Alves2,!Conceição!Lopes1!and!João!Raposo2!
!
(1)Department! of! Hematology! and! Bone! Marrow! Transplantation,! Centro! Hospitalar! Universitário!
Lisboa!Norte,!Lisbon,!Portugal,!(2)Department!of!Hematology!and!Bone!Marrow!Transplant,!Hospital!
Santa!Maria,!Centro!Hospitalar!Universitário!Lisboa!Norte,!Lisbon,!Portugal!
!
Introduction:! Mantle! Cell! Lymphoma! (MCL)! is! a! mature! BJcell! neoplasm! with! two! distinct!
variants!recognized!by!the!WHO!2016!lymphoma!classification:!the!aggressive!classical!MCL!
(cMCL)!and!the!more!indolent!leukemic!nonJnodal!variant.!Recently,!a!new!clinical!variant!
was! proposed! that! comprises! patients! with! MCL! presenting! with! only! extranodal! site!
involvement! or! predominant! extraJnodal! disease! with! minimal! locoregional!
lymphadenopathy!–!Mantle!Cell!Lymphoma!of!Mucosa!Associated!Lymphoid!Tissue!(MALTJ
MCL).!It!seems!to!have!a!more!favourable!prognosis!and!indolent!course!than!classical!nodal!
type!and!therefore!should!be!recognized.!
Case!Report:!We!present!the!case!of!a!70!yearsJold!man!with!ECOG!0!and!personal!history!of!
splenectomy! after! blunt! abdominal! trauma.! He! presented! with! mild! lymphocytosis! for! 15!
years!before!he!had!a!colonoscopy!for!colorectal!cancer!screening.!Mucosal!inflammation!on!
the!rectum!was!visualized!endoscopically.!Multiple!biopsies!were!performed!and!pathology!
examination!revealed!MCL!(immunohistochemistry!positive!for!CD20,!CD5!and!Cyclin!D1,!kiJ
67!40%)!without!IgVH!somatic!hypermutation.!PETJScan!showed!celiac,!gastric,!mesenteric!
and!iliopelvic!ganglia!uptake!(SUV!between!2.4!and!4.8)!and!diffuse!increased!gastric!uptake,!
with! higher! SUV! at! the! gastric! fundus! (maximum! 6.1);! other! cardiophrenic! and! axillary!
ganglia! with! maximum! SUV! of! 2.9! but! inferior! to! 2! cm! long! were! reported.! Gastric!
endoscopy! revealed! thickening! of! gastric! folds! and! irregular! granular! appearing! duodenal!
mucosa;!multiple!biopsies!documented!infiltration!of!duodenum!and!gastric!antrum!by!MCL.!
Bone!marrow!biopsy!showed!no!infiltration!and!although!lymphocytosis!was!present,!flow!
cytometry! was! negative! for! MCL.! The! patient! was! diagnosed! with! MCL! with! predominant!
extranodal!disease!(MALTJMCL),!Ann!Arbor!stage!IV!and!MIPIb!highJrisk.!Meanwhile!he!had!
a! transient! ischemic! attack! in! consequence! of! a! 90%! stenosis! of! the! right! internal! carotid!
artery,!which!was!successfully!treated!with!programmed!endarterectomy.!Nine!months!after!
the! diagnosis,! he! was! still! asymptomatic,! but! we! decided! to! start! treatment! based! on! the!
extension! of! gastrointestinal! involvement! and! risk! of! local! complications.!
Chemoimmunotherapy! with! 6! cycles! of! RJCHOP! followed! by! maintenance! with! rituximab!
was!uneventful!and!complete!response!was!achieved.!
Conclusions:!MALTJMCL!with!involvement!of!upper!and!lower!gastrointestinal!tract!can!be!
difficult!to!distinguish!from!gastrointestinal!tract!involvement!in!cMCL.!Although!MALTJMCL!
has! been! described! as! an! indolent! variant! with! better! prognosis! that! shouldn’t! be!
overtreated,!more!studies!are!needed!to!guide!treatment!decision,!especially!in!the!case!of!
advanced!stage!MALTJMCL.!
! !