POSTER'24'
LOW'GRADE'NONIHODGKIN'LYMPHOMA'AND'HODGKIN'LYMPHOMA:'
SEQUENTIAL'OCCURRENCE,'CONCOMITANT'DIAGNOSIS,'OR'
TRANSFORMATION.'A'RARE'PHENOMENON'
'
Ana!Carolina!Freitas1,!Raquel!Rodrigues1!and!Maria!Gomes!da!Silva2!
!
(1)Instituto! Português! de! Oncologia! de! Lisboa,! Francisco! Gentil,! Lisboa,! Portugal,! (2)Instituto!
Português!de!Oncologia!de!Lisboa!Francisco!Gentil,!Serviço!Hematologia,!Lisboa,!Portugal!
!
Background:! Histologic! transformation! of! follicular! lymphoma! and! chronic! lymphocytic!
leukemia!are!well!described!phenomena.!On!the!other!side,!the!transformation!of!marginal!
zone! lymphoma! (MZL)! and! other! lowJgrade! nonJHodgkin! lymphomas! (NHL)! is! a! poorly!
described! and! understood! event.! Sequential! occurrence! or! coJoccurrence! with! Hodgkin!
lymphoma! (HL)! is! exceptional.! The! mechanisms,! risk! factors! or! pathophysiology! of! such!
occurrence,!are!yet!to!be!described.!These!could!represent!a!clonally!related!situation!from!
a!common!precursor!or!an!incidental!and!unrelated!event.!Transformation!or!coJoccurrence!
of!MZL!and!HL!is!only!described!in!the!literature!in!the!form!of!rare!case!reports.!!
Objectives! and! Methods:! We! conducted! a! retrospective! analysis! of! patients! with! the!
diagnosis! of! lowJgrade! NHL! and! subsequent! or! simultaneous! diagnosis! of! HL! in! a! single!
center.!
Results:!We!collected!the!clinical!data!of!4!patients!along!the!last!17!years,!with!a!median!
age!of!63,5!(51J67)!yo;!3!were!female.!2!patients!had!the!initial!diagnosis!of!MZL!(1!nodal,!1!
extranodal),!and!one!of!them!concomitant!with!HL.!One!patient!was!initially!diagnosed!with!
lowJgrade! NHL! NOS! and! the! other! with! lymphoplasmacytic! lymphoma.! All! had! advanced!
stage!(III/IV)!disease!at!diagnosis,!75%!without!B!symptoms,!and!50%!with!elevated!LDH.!The!
patient!with!simultaneous!diagnosis!received!treatment!with!RJCVP!taking!into!account!that!
the! HL! was! nodular! lymphocyte! predominant,! subtype! D.! The! remaining! 3! received! RJ
bendamustine!or!RJCVP!for!NHL,!with!partial!response.!The!median!time!from!the!diagnosis!
of!lowJgrade!NHL!to!the!diagnosis!of!HL!was!48!months.!In!none!could!clonal!relationships!
be! investigated.! With! a! median! followJup! of! 91! months! from! the! diagnosis! of! the! first!
lymphoma:!1!patient!died!due!to!a!solid!tumor!and!the!other!are!still!alive!and!in!complete!
response!after!treatment!for!HL.!!
Conclusions:!This!clinical!series!demonstrates!the!existence!of!a!rare!phenomenon.!Indolent!
lymphomas!are!incurable!neoplasms,!requiring!prolonged!followJup.!Relapses!are!common!
and! histological! transformation! and! discordant! histologies! should! be! considered,! even!
without! clinical! suspicion,! and! call! for! biopsies.! Further! research! will! be! required! to!
understand! the! underlying! mechanisms! and! the! molecular! relationship! between! these!
entities.!
! !