MACROGLOBULINEMIA.WALDENSTRÖM.–.A.SINGLE.CENTRE.EXPERIENCE.
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Cátia% Sol% Reis,% Patrícia% Silva,% Eliana% Aguiar,% Rui% Bergantim,% Inês% Carvalhais,% Ana% Carneiro,% Jorge%
Cancela%Pires%and%Fernanda%Trigo%
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Clinical1Haematology1Department1of1Centro1Hospitalar1e1Universitário1de1São1João,1Porto,1Portugal1
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Introduction:%Macroglobulinemia% Waldenström% (MW)% is% a% rare,% indolent% mature% BUcell%
neoplasm% characterized% by% lymphoplasmacytic% cells% proliferation% associated% with% a%
monoclonal% immunoglobulin% M% protein.% Important% clinical% features% include% cytopenias,%
hepatosplenomegaly,% lymphadenopathy,% neuropathy% and% hyperviscosity.% Many% patients% do%
not%require%immediate%therapy%and%current%treatment%is%aimed%at%improving%quality%of%life%
and%not%to%complete%eradicate%the%disease.%
Objectives:%Evaluate% epidemiological% and% clinical% characteristics% of% patients% diagnosed% with%
MW%in%a%single%centre%and%analyse%negative%predictive%factors%of%survival%
Methods:%Single% centre% retrospective% analysis% of% MW% patients% diagnosed% between%
January/2009%and%December/2019%
%
Results:%Identified% 59% patients:% median% age% 73UyearsUold% 54U89,% 67.8%% male,% 100%%
Caucasian%and%none%had%family%history%MW.%Median%followUup%47%months%4U133.%
%
Most% common% clinical% manifestations:% anaemia% (55.9%),% lymphadenopathies% (40.7%),%
splenomegaly% (40%)% and% B% symptoms% (22%).% Plasmapheresis% was% performed% in% 4% patients.%
Median% IPSSUMW% at% diagnosis% was% 2% 0U4.% MYD88% and% CXCR4% mutations% were% not%
investigated.%
Treatment%was%carried%out%in%37%patients%(62.7%)%with%median%time%between%diagnosis%and%
treatment% initiation% of% 0% months% 0U62.% In% first% line% DRC% in% 21% patients% (56.8%),%
Chlorambucil+Prednisolone% 9% patients% (24.3%),% Rituximab% monotherapy% 3% patients% (8.1%)%
and% other% schemes% 4% patients% (RUCHOP,% RUCVP,% BDR).% Global% response% with% first% line%
treatment% was% 67.6%% with% only% 1% patient% achieving% a% complete% response,% 2% patients% had%
stable%disease%and%6%patients%(16.2%)%progressive%disease%(PD).%
The% patients% that% responded,% only% 2% needed% second% line% treatment,% both% achieving% partial%
remission%with%DRC,%without%the%need%of%subsequent%treatments.%
Patients%with%PD%were%treated%with%intensified%and%varied%second%line%options,%including%RU
CHOP,%RUESHAP,%DRC,%BDR%and%Ibrutinib,%with%all%patients%achieving%partial%response%except%1%
that% maintaining% PD.% That% patient% needed% 4% lines% of% treatment% (RUCHOP»RUESHAP»BRD»RU
Bendamustine)%to%achieve%a%partial%response.%
During%the%course%of%the%disease,%2%patients%had%histologic%transformation%into%an%aggressive%
lymphoma%(DLBC%and%plasmablastic%lymphoma).%
Currently,% 71.2%% patients% are% alive% (73%% overall% survival% at% 3% years)% and% 23.5%% of% deaths%
were%attributable%to%the%disease.%
On%multivariate%analyses,%it%was%identified%ECOG%PS%≥2%(p=0.015)%and%IPSSUMW%≥3%(p=0.012)%
at%diagnosis%to%have%negative%impact%on%survival.%
Conclusions:% MW% is% an% indolent% and% manageable% disease% with% currently% available%
treatments.% As% a% disease% characteristic% of% the% elderly,% the% median% survival% is% shorter%
compared% with% young% patients.% However,% a% significant% proportion% die% due% to% reasons%