POSTER'10'
A'55IYEARIOLD'MAN'WITH'A'DIAGNOSIS'OF'BLASTOID'MANTLE'CELL'
LYMPHOMA'ADMITTED'WITH'DYSPNOEA'AND'DISORIENTATION'
'
Pedro!Baptista,!Pedro!Chorão,!Ricardo!Pinto,!Rui!Bergantim!and!Fernanda!Trigo!
!
Centro!Hospitalar!e!Universitário!de!São!João,!Porto,!Portugal!
!
Introduction!
The!blastoid!variant!of!mantle!cell!lymphoma!(MCL)!is!an!uncommon!aggressive!form!of!BJ
cell! neoplasm! with! a! high! proliferative! index! and! poor! clinical! outcome.! Its! blastic!
appearance! is! associated! to! a! higher! degree! of! central! nervous! system! (CNS)! involvement!
and!extraJnodal!disease,!in!addition!to!the!typical!features!seen!on!classical!MCL.!
Case'report!
We!report!the!case!of!a!55JyearJold!man!diagnosed!with!stage!IVJA!MCL!after!initial!routine!
laboratory! tests! noted!de! novopancytopenia.! Marrow! histology! showed! neoplastic! cells! of!
variable!sizes,!including!intermediate/large!blastoid!CD10Jpositive!cells,!matching!the!CD10J
positive! clone! found! on! blood! immunophenotyping! (Table! 1).! The! patient! scored! an!
intermediate! risk! on! MCL! international! prognostic! index! and! started! treatment! with!
rituximab,! hyperfractionatedJcyclophosphamide,! vincristine,! doxorubicin! and!
dexamethasone! (RJhyperJCVAD)! alternating! with! rituximab,! highJdose! methotrexate! and!
cytarabine,!for!further!consolidation!with!autologous!stem!cell!transplantation.!
On! hospital! readmission! for! the! second! cycle! of! chemotherapy,! the! patient! complained! of!
progressive!dyspnoea!since!the!previous!week.!On!examination,!tachycardia!and!tachypnoea!
were! present,! as! were! jugular! venous! distension! and! lower! extremity! oedema.! Laboratory!
tests!were!unremarkable,!but!the!ECG!revealed!electrical!alternans!and!reduced!amplitude!
of!QRS!complexes!(Figure!1).!A!complementary!chest!radiograph!showed!enlargement!of!the!
cardiac!silhouette!(Figure!2A).!Altogether,!these!findings!were!consistent!with!a!pericardial!
effusion,! with! cardiac! tamponade! physiology! confirmed! on! echocardiographic! assessment.!
Pericardiocentesis! allowed! drainage! of! 1! 500! ml! of! serohematic! fluid! displaying! the! same!
neoplastic!CD10Jpositive!clone!of!the!previously!diagnosed!blastoid!MCL!(Table!1).!
After!haemodynamic!recovery,!and!during!preparation!for!systemic!chemotherapy,!insidious!
temporospatial!disorientation!was!noted.!Cerebral!magnetic!resonance!imaging!showed!no!
significant! changes,! but! lumbar! puncture! revealed! mononuclear! leucocytosis! on! the!
cerebrospinal!fluid!(CSF),!with!a!CD10Jpositive!population!on!immunophenotyping!analysis!
(Table!1).!Confirmation!of!CNS!involvement!prompted!initiation!of!intrathecal!methotrexate,!
cytarabine! and! dexamethasone! until! CSF! clearance.! The! patient! progressed! to! full!
neurological! recovery! and! was! discharged! after! completion! of! the! second! cycle! of!
chemotherapy.!
Discussion!
This! case! highlights! the! contribution! of! frequent! CNS! involvement! and! extraJnodal! disease!
spread! for! worsening! prognosis! in! patients! with! blastoid! MCL.! Further! research! on! the!
characteristics! of! this! distinctive! lymphoma! subtype,! including! the! trend! for! unusual! CD10!
expression,! along! with! additional! clinical! trials! specifically! directed! at! these! patients,! are!
needed! to! build! consensual! guidelines! for! CNS! prophylaxis! and! systemic! treatment.! This!
might!be!particularly!important!in!intermediateJrisk!patients,!where!therapeutic!approaches!
are!even!more!illJdefined.!