POSTER'34'
REFRACTORY'PRIMARY'EFFUSION'LYMPHOMA'IN'AN'ELDERLY'
IMMUNOCOMPETENT'PATIENT'TREATED'WITH'BRENTUXIMAB'VEDOTIN'
'
Francisca!Miranda,!Cláudia!Claudino,!Filipa!Mousinho,!Lénia!Rodrigues,!Tatiana!Mendes,!Ana!Jorge,!
Alice!Reichert!and!Celina!Afonso!
!
Hospital!de!São!Francisco!Xavier,!Centro!Hospitalar!de!Lisboa!Ocidental,!Lisbon,!Portugal!
!
INTRODUCTION!
Primary!effusion!lymphoma!(PEL),!a!rare!subtype!of!diffuse!large!BJcell!lymphoma,!accounts!
for!~4%!of!Human!Immunodeficiency!Virus!(HIV)!associated!NonJHodgkin!Lymphomas!and!<!
1%! of! nonJHIV! related! lymphomas.! Caused! by! Human! herpesvirus! 8! (HHV8)! infection! in!
immunocompromised! patients,! most! commonly! HIVJinfected! individuals,! but! also! in! organ!
transplant!recipients!or!chronic!hepatitis!C.!
Presents!as!an!effusion!in!body!cavities,!usually!without!lymphadenopathy!or!tumor!masses.!
No! optimal! treatment! exists,! and! the! prognosis! is! poor,! with! an! average! survival! of!
approximately! 10! months.! Frontline! therapy! relies! on! combined! chemotherapy! regimens,!
however! most! patients! relapse! within! 6J8! months.! Since! PEL! is! typically! CD30! positive,!
Brentuximab!Vedotin!(BV)!may!be!considered!as!a!therapeutic!option,!although!there!aren't!
clinical!trials!of!BV!treatment!in!PEL.!
CLINICAL'CASE!
77! yearJoldJmale,! ECOG! 1! with! past! medical! history! of! arterial! hypertension,! depression,!
gastritis.!Admitted!in!the!Emergency!Department!in!April/2019,!presenting!with!progressive!
and! worsening! fatigue.! Chest! xJray! revealed! moderate! right! pleural! effusion! (PE).!
Immunophenotype!of!pleural!fluid!(PF)!showed!pathological!lymphocytes!positive!for!CD30,!
CD38,! CD4,! negative! for! CD20,! CD3,! CD5.! Histology! with! atypical! large! lymphocytes,!
immunohistochemistry!CD30+,!HHV8+,!CD20J,!CD3J.!HIV!and!other!viral!serologies!negative.!
CT! scan! detected! no! additional! effusion,! masses! or! lymphadenopathy.! No! marrow!
infiltration! by! lymphoma.! In! May/2019! CHOP! protocol! was! started,! and! after! 3! cycles,! PE!
radiological!reduction!and!PF!drainage!independence!were!achieved.!In!August/2019,!after!4!
cycles,! worsening! fatigue! and! dyspnea! developed.! CT! scan! detected! extensive! right! PE!
without! other! cavity! effusion.! In! November/2019,! 2nd!line! treatment! with! BV! was! started!
with! improvement! of! PE! after! 3! cycles.! At! last! followJup! on! August/2020,! he! underwent! a!
total! of! 13! cycles,! without! respiratory! symptoms,! clinical! or! imaging! evidence! of! disease!
progression.!
DISCUSSION!
Due! to! the! rarity! of! this! disease,! evidence! supporting! optimal! treatment! of! PEL! is! lacking,!
particularly! in! relapsed! and! refractory! disease.! Available! treatment! options! are! associated!
with! poor! results,! so! novel! approaches! are! warranted.! BV! showed! efficacy! in! PEL! in!
preclinical!models.!However!additional!data!is!required,!clarifying!the!value!of!BV!in!PEL.!
This! case! illustrates! a! case! of! PEL! in! an! immunocompetent! patient,! initially! responding! to!
chemotherapy,! but! becoming! refractory! after! 4! cycles.! Following! 13! cycles! of! 2nd!line! BV!
monotherapy,! no! sign! of! disease! progression! or! unacceptable! treatment! toxicity! were!
noticed.! Although! representing! an! isolated! case,! it! may! contribute! as! clinical! evidence! to!
support!the!use!of!BV!for!PEL.!
! !
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