POSTER'12'
ANAPLASTIC'LARGE'CELL'LYMPHOMA'IN'PAEDIATRICS'(ALCL)'–'
RETROSPECTIVE'STUDY'OF'A'CENTRAL'HOSPITAL'
'
Inês! Brito1,! Graça! Sousa2,! Ana! Pinelas3,! Nuno! Gomes4,! Sílvia! Silva5,! Cátia! Sousa5,! Cristiana! Couto5,!
Filipa!Almeida5,!Catarina!Macedo!Sousa5,!Íris!Maia5,!Marta!Almeida5,!Vítor!Costa5,!Tereza!Oliva5!and!
Ana!Ferreira5!
!
(1)Department! of! Clinical! Haematology! of! Centro! Hospitalar! Universitário! de! São! João,! EPE,! Porto,!
Portugal,! (2)Department! of! Paediatrics! of! Hospital! Central! do! Funchal,! Madeira,! Funchal,! Portugal,!
(3)Department! of! Medical! Oncology! of! Instituto! Português! de! Oncologia! do! Porto,! EPE,! Porto,!
Portugal,!(4)Department!of!Dermatovenereology!of!Centro!Hospitalar!Universitário!de!São!João,!EPE,!
Porto,! Portugal,! (5)Department! of! Paediatrics! of! Instituto! Português! de! Oncologia! do! Porto,! EPE,!
Porto,!Portugal!
!
Objectives'Assessment!of!demographic,!clinical,!treatment!and!outcome!details!on!patients!
with!diagnosis!of!ALCL,!at!a!central!hospital.!
Methods'Retrospective!observational!analysis!of!all!cases!of!ALCL!younger!than!18!years!of!
age! presenting! between! August! 2009! and! August! 2020! with! an! immunohistologically!
confirmed!diagnosis!of!ALCL.!
Results'ALCL! is! primarily! a! paediatric! tumour,! accounting! for! 15%! of! all! childhood! nonJ
Hodgkin! lymphomas.! In! children,! most! ALCL's! exhibit! a! t(2;5)! translocation,! which! can! be!
detected!by!anaplastic!lymphoma!kinase!(ALK)!antibodies.!ALCL!often!presents!in!advanced!
clinical! stages.! In! published! paediatric! series,! the! analysis! of! prognostic! factors! has! been!
limited! to! date! because! of! the! small! number! of! patients,! but! many! studies! described! B!
symptoms,!mediastinal!mass,!spleen,!liver!or!lung!involvement,!skin!lesions,!high!stage!in!the!
St! Jude! or! Ann! Arbor! classifications,! and! elevated! LDH! as! increased! risk! of! progression! or!
relapse.! Primary! cutaneous! ALCL! appears! to! have! a! different! pathogenesis! and! better!
prognosis!than!does!systemic!ALCL,!presenting!as!one!or!more!skin!tumors,!usually!localized.!
It!remains!one!of!the!most!curable!cancers!in!the!paediatric!setting,!because!it!is!relatively!
chemoJsensitive.!
We!collected!data!from!seven!patients!with!a!median!followJup!of!74!months.!Age!ranged!
from!4!to!11!years!(median!9!years),!4!were!male!(57%).!Clinical!features!are!described!in!
Table!1.!
Six! patients! were! treated! according! to! protocol! ALCL99! and! 83%! achieved! a! complete!
remission!(CR).!The!one!patient,!who!did!not!achieve!a!CR,!had!a!partial!response!(PR)!and!
underwent! relapse! protocol! chemotherapy! (ALCL! Relapse)! and! consolidation! with!
autologous!stem!cell!transplantation,!resulting!in!CR!at!the!end!of!second!line!therapy.!One!
patient!with!primary!cutaneous!ACLC!had!no!criteria!for!treatment!and!remained!in!watch!
and!wait!strategy.!All!of!six!treated!patients!did!central!nervous!system!prophylaxis.!Overall,!
progression! free! survival! at! 18! months! was! 86%! and! overall! survival! was! 100%.! None!
developed!a!secondary!malignancy!during!follow!up.!
Conclusion'In!this!study!we!describe!seven!patients!who!were!confirmed!to!have!ALCL.!The!
median!age!at!diagnosis!was!9!years.!Despite!its!aggressive!presentation!and!the!need!for!
intensive! chemotherapy! regimens,! ALCL! has! good! response! rates! in! the! paediatric! age.!
Although! the! low! number! of! patients! do! not! allow! firm! conclusions,! our! results! are! in!
accordance! with! the! literature.! Given! the! longJterm! survival! of! these! patients,! it! is!
imperative!to!monitor!secondary!neoplasms.!
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