MULTIFOCAL'EXTRAINODAL'MARGINAL'ZONE'LYMPHOMA'PRESENTING'
AFTER'TWO'YEARS'OF'REUMATHOID'ARTHRITIS'AND'SECONDARY'SJÖGREN’S'
POSTER'46'
SYNDROME'DIAGNOSIS'
'
Ana!Vagos!Mata1,!Nikita!Khmelinskii2,3,!Daniela!Alves1,!Catarina!Quadros4,!Cristina!Ferreira4!and!João!
Raposo1!
!
(1)Department!of!Hematology!and!Bone!Marrow!Transplant,!Hospital!Santa!Maria,!Centro!Hospitalar!
Universitário! Lisboa! Norte,! Lisbon,! Portugal,! (2)Rheumatology! Department,! Hospital! Santa! Maria,!
Centro! Hospitalar! Universitário! Lisboa! Norte,! Lisbon,! Portugal,! (3)Rheumatology! Research! Unit,!
Instituto! de! Medicina! Molecular,! Faculdade! de! Medicina,! Universidade! de! Lisboa,! Lisbon,! Portugal,!
(4)Department! of! Pathology,! Hospital! Santa! Maria,! Centro! Hospitalar! Universitário! Lisboa! Norte,!
Lisbon,!Portugal!
!
Objective:! To! highlight! the! rarity! of! marginal! zone! lymphoma,! especially! in! its! extraJnodal!
and! multifocal! form,! and! to! focus! on! the! therapeutic! challenge! of! treating! patients! with!
multiple!comorbidities.!
Methods:!Retrospective!review!of!a!clinical!case,!based!on!clinical!registries!and!laboratory!
findings.! The! diagnosis! was! performed! after! gastric! and! pulmonary! biopsies.! CTJscan! and!
upper! endoscopy! were! complementary! techniques! used! at! diagnosis! and! response!
evaluation.!
Results:!We!present!a!case!of!a!46JyearJold!woman!diagnosed!with!rheumatoid!arthritis!with!
secondary! Sjögren’s! syndrome! in! 2017.! She! was! initially! treated! with! prednisolone,!
methotrexate,! and! hydroxychloroquine.! After! the! diagnosis! of! lymphocytic! interstitial!
pneumonia,!by!biopsy!of!pulmonary!nodular!infiltrates,!therapy!with!rituximab!was!initiated.!
By! April! 2018,! she! developed! a! biJclonal! gammopathy! (IgA! and! IgM)! and! was! referred! for!
Hematology!consultation.!Serum!IgA!and!IgM!slowly!increased!and,!by!the!end!of!2019,!she!
presented!5224!mg/dL!IgM!kappa,!614!mg/dL!IgA!kappa,!and!12%!of!plasmocytes!without!
monoclonality! in! the! bone! marrow! examination.! A! revaluation! CTJscan! of! pulmonary!
infiltrates!revealed!an!increase!in!the!size!of!some!nodules,!particularly!one!of!the!lingula,!
with! 17.6! x! 15! mm;! also,! there! was! thickening! of! the! posterior! and! anterior! walls! of! the!
gastric! body.! Upper! endoscopy! showed! an! atrophic! gastric! body! mucosa.! After! lung! and!
gastric! biopsies! a! diagnosis! of! stage! IV! (Lugano! classification),!H.! pylori!negative,! mucousJ
associatedJlymphoidJtissue! lymphoma,! was! established.! This! patient! was! on! isoniazid!
prophylaxis! for! latent! tuberculosis,! had! seroconverted! hepatitis! B! (positive! antiJHbc),! and!
needed! supplemental! oxygen! therapy! (1L/min.! by! nasal! cannula)! during! minor! efforts.!
Considering!the!need!for!treatment!and!all!comorbidities!despite!the!young!age,!she!started!
rituximab! plus! chlorambucil,! with! good! tolerability.! Six! cycles! were! administered! from!
January! through! June! of! 2020! with! entecavir! prophylaxis! for! hepatitis! B! virus! reactivation.!
There!was!a!significant!clinical!improvement:!the!patient!suspended!oxygen!support!and!has!
no!dyspnea!on!exertion.!We!are!currently!waiting!for!the!response!evaluation!exams.!
Conclusions:!On!a!fragile!patient!with!multiple!comorbidities!we!achieved!a!partial!response!
without! any! infectious! complications! or! need! for! inpatient! care.! Treatment! was!
administered!with!no!delays!during!the!COVIDJ19!outbreak.!More!intensive!chemotherapy!
schemes!could!have!led!to!major!toxicity!and!adverse!events!in!a!difficult!to!manage!patient.!
After!the!clinical!improvement,!and!still!waiting!for!full!response!evaluation!exams,!remains!
the!question!of!whether!to!watch!&!wait!or!to!start!maintenance!treatment.! !
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