rev bras hematol hemoter. 2015;37(2):127–129
w w w . r b h h . o r g
Revista
Brasileira
de
Hematologia
e
Hemoterapia
Brazilian
Journal
of
Hematology
and
Hemotherapy
Case
Report
Burkitt’s
lymphoma
successfully
treated
in
pregnancy
Alan
Lúcio
Alves
Inácio
Júnior
∗,
Beatriz
Fernandes
Rocha,
Luís
Fábio
Barbosa
Botelho
UniversidadeFederaldaParaíba(UFPB),JoãoPessoa,PB,Brazil
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Articlehistory:
Received9May2014 Accepted30June2014
Availableonline21November2014
Introduction
Neoplastic diseases affect about 1:1000 women during pregnancy.1Thisincidencevariesaccordingtoregion,ageand
thepresenceorabsenceofpre-invasivediseases.2
Lymphomais the fourth mostdiagnosed cancer inthis period,behindbreasttumors,cervicalcancerandmelanoma.3
Itsfrequencyhasrisenwiththetendencyinmodernsociety ofpostponingpregnancy.4
The most common lymphoma in pregnant patients is Hodgkin’s lymphoma, since its incidence spike coincides withthefemalefertile age.Anylinkbetweennon-Hodgkin lymphoma and pregnancy has still not been elucidated, withfewcasereportsbeingfoundinthemedicalliterature worldwide.3,4
Burkitt’slymphoma isa very aggressivegerminal B-cell lymphoma,frequentlypresentingextranodal sites.Itisthe secondmostcommonlymphomainchildren,butitisveryrare inadults.Threeclinicalvariantsareknown:endemic,sporadic andimmunodefficiency-related.5
∗ Correspondingauthorat:UniversidadeFederaldaParaíba(UFPB),CampusI,JardimUniversitárioS/N,CasteloBranco,58051-900João
Pessoa,PB,Brazil.
E-mailaddress:[email protected](A.L.A.InácioJúnior).
Inthesporadicform,theprimarylocationofthedisease involves the abdomen in 80% and the mandible in14% of patients. In the endemicform, themandibular and maxil-laryareinvolvedin60%ofpatients,abdominalcommitment in58%ofcases,followedbythecentralandparaspinal ner-voussystems.Animportantgeographicdifferencebetween thesetwoformsistheassociationofEpstein–Barrvirus(EBV) withtheendemicform.However,only15–20%ofBurkitt’s lym-phomasseeninEuropeandintheUSAareassociatedwith EBV.ThefrequencyofthisassociationinSouthAmericaseems intermediateand,inBrazil,afrequencyof70%forEBV asso-ciatedwithBurkitt’slymphomahasbeenreported.5
Burkitt’s lymphoma was the first identified neoplasm whosepathogenesisinvolvedachromosomaltranslocationas demonstratedbymolecularbiology.Thismutationinvolvesa translocationbetweenthedistalpartofthelongarmof chro-mosome8andchromosome14[t(8;14)(q24;q32)].Asaresultof thisrearrangement,themycproto-oncogeneinchromosome 8istranslocatedtotheimmunoglobulinheavychainlocusin chromosome14.6
http://dx.doi.org/10.1016/j.bjhh.2014.11.002
128
revbrashematolhemoter.2015;37(2):127–129Case
report
A 25-year-old female patient born in Santa Rita, state of Paraíbaconsultedwitha slightstingingabdominalpain in thelefthypochondriacandlumbarregions,associatedwith diarrhea and melena forthree months. The resultof a b-HCGtest,performedbecauseofamenorrhea,waspositive.The obstetricultrasonography(USG)revealedafour-weektwo-day pregnancy.Afteronemonth,therewasanincreaseinthepain, associatedwithnauseaandweightloss.AnewobstetricUSG, performedduring prenatal care,showed amorphologically normalfetus(12weeks),inadditiontorightcolonthickening inthemother,withanextensive(5.0cm×3.0cm)
vascular-izedhypoechogenicformationnexttothehepaticangle.The patientwasaskedtourgentlyseekagastroenterologist.
With significant worsening of the abdominal pain and acuteexacerbationofthecondition,thepatientwenttothe HospitalUniversitárioLauroWanderley,whereshewas admit-tedtoperformmoretests.Acomputertomographyshoweda softtissuemassintheupperthirdpartoftheabdomen,with air-fluidlevel,and dilationofanteriorintestinalloops with partialobstruction(Figures1and2).
A further abdominal USG revealed an extensive (10.6cm×5.4cm)echo-complexmassintheright
hypochon-drial region, with a heterogeneous aspect (solid/cystic). A colonoscopy revealed a vegetative lesion of about 15cm occupyingaround90%oftheintestinallumen.
Thepatientwassubmittedtoenterectomyand typhlec-tomy,inadditiontorightsegmentalcolectomywithprimary latero-lateralileocolonicanastomosis.Surgerywascompleted withoutcomplicationsandthepatientevolvedwellafterthe procedure,withoutcomplaintsofbleedingorpaininthelower abdomen. Anevaluationofthe fetal was madeconfirming preservedvitality.Thepatientwasprescribednatural proges-terone(400mg/day)inordertokeeptheuterusquiescent.
Themassremovedduringsurgerywassubmittedto anato-mopathologicalevaluationrevealingmediumandlargecell non-Hodgkin lymphoma. Immunohistochemistry identified
Figure1–Heterogeneousmassoccupyingalmostallofthe ascendingandtransversecoloniclumenwithdilationand signsofdistalobstruction.
Figure2–Softtissuemassintheupperthirdofthe abdomenwithair-fluidlevel,dilationoftheanterior intestinalloopsandpartialobstruction.
featuresofBurkitt’slymphoma.Afterdischarge,thepatient wasreferredforonco-hematologicalandobstetriccare.
Thestagingofthelymphomarevealedisolatedabdominal disease,withnobonemarroworcerebrospinalfluid involve-ment; it was classified as Murphy II-B. A 21-day regimen ofrituximab IV(375mg/m2 – Day 1),cyclophosphamideIV
(500mg/m2 perday–Day 1–3),vincristineIV(2mg–Day1
andDay8),doxorubicinIV(60mg/m2–Day1),oralprednisone
(60mg/m2 – Day 1–5) (COPAD-R) was started following the
LMB-96protocol.Moreover,prophylaxisofthecentralnervous systemwascarriedoutusingintrathecalchemotherapy: dexa-methasone(2mg)andmethotrexate(12mg–Day2andDay 5).7–9
The patient was advised about the aggressiveness of the condition and the possible risks to the fetus during chemotherapy, but chose not to interrupt pregnancy. The patienthadagoodevolutionwithclinicalimprovement.
Duringobstetriccare,anothermorphologicalobstetricUSG revealed anon-ectopic pregnancywith anechographic age of 19.1 weeks – in addition to an abdominal USG, which showedasolidabdominalmass(8.6cm×10.8cm)inamedial
and slightly left position, which was heterogeneous with irregularedges.AnUSGperformedthefollowingweekshowed anon-ectopicpregnancywithechographicageof20.3weeks and preserved fetal morphology, and a solid abdominal mass(6.6cm×3.6cm×5.0cm),identicaltotheonepreviously
described,indicatingthattherewasadecreaseinsizeafterthe beginningofchemotherapy.
Afterfourcyclesofchemotherapy,thepatientevolvedwith anewacuteabdominalcondition.Laparotomyshoweda per-forationintheproximaljejunum,andsoanotherenterectomy andurgentcaesariansection(33rdweekofpregnancy)were performed. Ahealthy female childof1815gwas delivered. Thesegmentremovedinthesecond enterectomywassent foranatomopathologicalstudy,whichshowednodisease.A computertomographyafterbirthwasnormal.
revbrashematolhemoter.2015;37(2):127–129
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Discussion
Burkitt’s lymphoma, although very aggressive, is highly treatable.10 The objective of treatment is to eliminate the
largestnumberofmalignantcellspossibleandinduce com-plete remission, in other words, the disappearance of all evidence of the disease. Patients with aggressive and fast growing lymphomasare frequently treatedwith combined chemotherapy.Intensivechemotherapywithmultipledrugs may be very effective against aggressive lymphomas. The prognosisofBurkitt’slymphomadependsontheextentofthe diseaseandthetimeintervalbetweenthefirstcomplaintsand diagnosis.Thedisease-freesurvivalrateisbetween75%and 85%inadults.6
There is no unique option for chemotherapeutic treat-mentofthiskindoflymphoma.Today,thechemotherapeutic treatmentconsistsinshort-durationintensiveregimensthat includeagentssuchasmethotrexate,cyclophosphamide, vin-cristine,doxorubicinandprednisone.Methotrexateisusedin mostofthebigcenters,butitishighlytoxicandis respon-sibleformostoftheriskduringpregnancy.Thetreatmentis complementedwithprophylaxisforinfections.Relapse some-timesoccursduringthefirstyear,andisaparticularlypoor prognosticsign.11
Thetreatmentofapregnantwomanwithcancerisalways adelicatedecision,sinceitinvolvesrisksforthemotherand child,thewishesofthepatientandtheopinionsofthe oncol-ogist,theobstetricianand the neonatologist.Thepotential effectsofantineoplasticagentstothefetusinclude immedi-ateeffects,suchasabortionandteratogenesiswithspecific damagetooneormoreorgans,andlateeffects,suchasdelay ingrowthandgonadaldysfunction.1
ThechoiceoftheCOPAD-RregimenfollowingtheLMB-96 protocol,7–9aimingatgreaterpracticalitywithfewerrisksfor
thepatient, wasverysuccessful,ensuringremissionofthe diseaseandcompletionofthepregnancy.
Therareoccurrenceoflymphomaduringpregnancy pre-ventslargeprospectivestudiesbeingperformedtoexamine issuesaboutdiagnosis,managementandtheoutcomes.The literatureaboutthesubjectispoorandbasedonretrospective analysis,andsofurtherstudiesarenecessary.
Conclusion
Theassociationofcancerandpregnancyisaveryrare con-dition.Duringalongtime,the occurrenceofcancerduring
pregnancy wasrelatedtotheideaofaveryaggressive dis-ease,withreservedprognosisandlittleperspectivetotreat. However,recently,therehavebeenreportsofcasesinwhich chemotherapywassuccessfullyusedinpregnantwomen.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
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