NK/T'NASAL'TYPE'LYMPHOMA:'A'VERY'RARE'AND'AGGRESSIVE'DISEASE'
POSTER'17'
'
Maria! Eduarda! Couto1,! Mário! Pimenta1,! Fabiana! Munoz1,! Catarina! Dantas! Rodrigues2,! Sara!
Petronilho3,!Isabel!Oliveira1,!Nelson!Domingues1,!Luisa!Viterbo1,!Angelo!Martins1,!Ilídia!Moreira1,!Ana!
EspiritoJSanto1,!Sérgio!Chacim1,!Cláudia!Moreira1,!Dulcineia!Pereira1,!Carla!Castro4,!Angelo!Oliveira4,!
Rui!Henrique3!and!José!Mariz1!
!
(1)OncoUhematology!Service,!Instituto!Português!de!Oncologia!do!Porto,!F.G.,!E.P.E.,!Porto,!Portugal,!
(2)Hematology! Service,! Centro! Hospitalar! de! Tondela! Viseu,! Viseu,! Portugal,! (3)Pathology! Service,!
Instituto! Portugues! de! Oncologia! do! Porto,! F.G.,! E.P.E.,! Porto,! Portugal,! (4)Radiotherapy! Service,!
Instituto!Portugues!de!Oncologia!do!Porto,!E.P.E,!Porto,!Portugal!
!
NK/T! nasal! type! lymphoma! (NKTCL)! is! a! rare! disease! that! commonly! involves! the! midline!
area,! mainly! the! nasal! cavity,! oral! cavity! and! pharynx,! causing! massive! and! necrotic! local!
lesions.!It!is!more!common!in!Asia,!being!extremely!rare!in!Europe.!EBV!infection!is!found!in!
the!majority!of!the!cases.!Chemotherapy!with!radiotherapy!(sometimes!with!central!nervous!
system/CNS! prophylaxis)! are! the! most! common! strategies! used,! but! the! overall! survival! is!
very!poor.!
A! retrospective! and! unicenter! study! was! performed! for! NKTCL.! The! clinical! files! of! 10!
patients! identified! with! NKTCL! between! 2008J2020! were! reviewed! until! september! 2020.!
One! of! the! cases! was! excluded! for! the! lack! of! information! (the! patient! was! treated!
previously!in!another!hospital).!
Nine! patients! were! analyzed:! 66.7%! males,! median! age! 60! years! 35J83,! 44.4%! were! in!
stage!IV!of!disease,!33.3%!were!in!stage!I!and!22.2%!were!in!stage!II.!EBV!was!found!in!the!
histology!of!88.9%!of!the!cases!and!in!the!serology!of!44.4%.!SNC!evaluation!was!positive!in!
one!patient.!
Nearly! 67%! of! the! patients! did! first! line! therapy! with! a! CHOPJscheme! (n=6;! 3! did!
radiotherapy;! 3! of! them! did! SNC! prophylaxis! with! methotrexate);! 22.2%! did! ACOB! +! SNC!
prophylaxis! with! methotrexate! +! radiotherapy;! 11.1%! did! ICE.! 55.5%! of! these! patients!
treated!with!first!line!achieved!a!complete!response.!The!progression!free!survival!after!the!
first!line!therapy!was!1!month!0J106.!Five!cases!did!second!line!therapy:!40%!of!them!with!
ICE! +! Alentuzumab,! 20%! with! ICE! +! SNC! prophylaxis! with! methotrexate,! 20%! with!
radiotherapy! and! 20%! with! LJAsparaginase! +! methotrexate! +! dexamethasone.! Three! cases!
did!a!third!line!therapy!with!Alentuzumab!+!Fludarabine!(33.3%)!or!Etoposide!+!Procarbazine!
+! Cyclophosphamide! +! Prednisolone! (33.3%)! or! Fludarabine! +! Cyclophosphamide! (33.3%),!
none! with! response.! Only! one! case! did! a! fourth! line! therapy! with! Etoposide! +!
Dexamethasone,!without!response.!
The!median!followJup!time!was!11!months!0J87,!the!median!event!free!survival!time!was!1!
month! 0J106.! 77.8%! of! the! cases! died! (n=7,! 6! of! them! with! disease).! Both! of! the! two!
patients!still!alive!are!in!response!(one!of!them!complete).!
This!casuistry!shows!a!population!highly!refractory!to!standard!treatments!(only!2!patients!
achieved!long!term!remission),!with!a!very!poor!prognosis!and!an!estimated!overall!survival!
of!few!months.!New!regimens!directed!to!gene!mutations!and!expression!abnormalities!are!
expected!to!improve!survival!in!such!an!aggressive!disease.!
! !