BrazJOtorhinolaryngol.2014;80(6):546---548
Brazilian
Journal
of
OTORHINOLARYNGOLOGY
www.bjorl.org
CASE
REPORT
Nasopharyngeal
Burkitt
lymphoma
as
an
early
AIDS
manifestation
夽
Linfoma
de
Burkitt
nasofaríngeo
como
manifestac
¸ão
inicial
de
Sida
Juliana
Gama
Mascarenhas
a,
Francisco
Araújo
Júnior
b,
Thiago
Villela
Bolzan
a,
Luis
Carlos
Gregório
a,
Eduardo
Macoto
Kosugi
a,∗aRhinologyDivision,EscolaPaulistadeMedicina,UniversidadeFederaldeSãoPaulo(UNIFESP),SãoPaulo,SP,Brazil
bDepartmentofOtorhinolaryngologyandHeadandNeckSurgery,EscolaPaulistadeMedicina,UniversidadeFederaldeSãoPaulo
(UNIFESP),SãoPaulo,SP,Brazil
Received20February2013;accepted30April2013 Availableonline3July2014
Introduction
Burkitt lymphoma (BL) is a highly aggressive type B non-Hodgkin’slymphoma(NHL)foundinendemic,sporadic,and immunodeficiency-relatedpresentations.Allofthemshow thesamemorphological,immunohistochemical,andgenetic characteristics, with different geographical and age dis-tributions and affected organs.1,2 The great majority of
sporadicandimmunodeficiency-relatedcases arefound in
theabdomen(60---80%),followedbyheadandneck,where
Waldeyer’s ring extranodal involvement is usual (palatine
tonsilandnasopharynx).1---3
This studyaimedtoreporta casein whichthe patient
presentedBLasanearly manifestationofacquired
immu-nodeficiencysyndrome(AIDS).
夽 Please citethis article as:Mascarenhas JG, Araújo Júnior F,
BolzanTV, Gregório LC,KosugiEM. Nasopharyngeal Burkitt lym-phomaas an early AIDS manifestation. Braz JOtorhinolaryngol. 2014;80:546---8.
∗Correspondingauthor.
E-mail:edumacoto@uol.com.br(E.M.Kosugi).
Case
report
R.F.C., a 44-year-old mixed-race male locksmith, born in
Bahia andliving inSão Paulo, presented to the
Otorhino-laryngologyEmergencyRoomwithableedingnosefollowed
by massive oral bleeding with hemodynamic instability,
requiringablood transfusion.Althoughpreviously healthy,
the patientrecounteda progressiveleftnasalobstruction
thathadbecomebilateralfourmonthsearlier,andwas
asso-ciatedwith ayellowish nasaldischarge and anosmia.The
conditionprogressedwithtrismus,dysphagia,bilateral
ear-ache,andleftorbitalpain.Nohearinglosswaspresent,but
hehadearfullness.Healsohadlost20kginthepasttwo
months andwasa 26 pack-yearssmoker, a social drinker,
andaformerintravenouscocaineuser.
Onphysicalexamination,hisgeneralstatuswasnormal,
andhehadapaleconjunctiva,an emaciatedappearance,
mouth breathing, trismus, and a left convergent
strabis-mus.Nasalendoscopyrevealedabilateralmucoiddischarge
andapale,friablelesionthatmadetheexaminationmore
difficult. Computed tomography and magnetic resonance
imagingofparanasalsinuses(Fig.1)showedanenlargement
oftheposteriorwallofthenasopharynxwithapost-contrast
enhancementprotrudingintothechoanae,nasalcavity,and
oropharynx. Bone destruction was noted at the sphenoid
http://dx.doi.org/10.1016/j.bjorl.2014.05.030
NasopharyngealBurkittlymphomaasanearlyAIDSmanifestation 547
Figure1 Computedtomographyofparanasalsinuses.(A)Coronalview,bonewindowand(B)axialview,softtissuewindow,with asofttissuedensitylesioninthenasopharynx,nasalcavity,andsinuses;magneticresonanceimagingofparanasalsinuses,(C)axial view,contrastT1-weightedimaging,(D)T2-weightedview,(E)T1-weightedsagittalviewwithanenlargementofnasopharyngeal posteriorwallandpost-contrastenhancementprotrudingintothenasalcavity;lesionmicroscopywith(F)massivelymphoidcell proliferation,withsubmucoushistiocytescontainingintracytoplasmicstainablebodieswith astarry skyappearance,(G)CD-20 positiveneoplasticcells,(H)CD3-negativeneoplasticcells,(I)virtually100%ofcellspositiveforKi67,acellproliferationmarker.
sinusfloor,andtherewasasymmetryinadjacent
parapha-ryngealfatplanes,withaneffacementontheleft.
Anendoscopicincisionalbiopsyofthenasalcavitylesions
and the hardened nasopharyngeal lesion was performed.
An exposed and engorged vessel was identified in the
nasopharynx,whichcouldexplaintheseverebleedingfrom
the mouth. Histopathological examination demonstrated
basophil cells with numerous lipid vacuoles,round nuclei
withdensechromatinandmultiplenucleoli,andstarrysky
diffuse infiltration pattern consistentwith classicBL. The
immunohistochemistry confirmed Burkitt B-cell NHL, and
waspositiveforCD20,CD3,CD10,Bc16,andki67;and
neg-ativeforBc12,TdT,CD99,CD56,andEBV.
One week following the biopsy, there was a tumor
enlargement,withproptosis,worseneddysphagia,
respira-tory distress, majorhealth statusworsening, andmelena.
Thepatientwasadmittedtothehospitalwithdehydration,
malnutrition, acute kidney failure, severe sepsis criteria,
andwiththepresenceofatumorlysissyndrome.Serological
investigationrevealedhumanimmunodeficiencyvirus(HIV)
infectionwithaCD4countof318.Bonemarrowexamination
showed 63% blasts, thus demonstrating Burkitt leukemia.
Afterproperclinicalsupport,aggressiveantiretroviral
ther-apy andEPOCH-R-based chemotherapy were initiated.He
underwent tracheostomy to secure the airway. After ten
daysofchemotherapy,hehadnonasalobstructionandhad
respondedfavorablytotreatment.
Discussion
BL may be the first AIDS-defining criterion, as this kind
oflymphoma oftenoccurswithhigh levelsofCD4 (>200),
that is, at an early stage of immunodeficiency with few
symptoms.1,2,4Indevelopingcountries,theconcurrent
diag-nosisofHIVandlymphomaoccursinuptohalfofallcases,
whereas the rate is substantially lower (around 10%) in
developedcountries,whichhighlightsthedifficultyofearly
HIVdiagnosisindevelopingcountries.4
Final
remarks
BLshouldbeincludedindifferentialdiagnosisof
nasopha-ryngealtumorsevenwithnopriorHIV diagnosis,asitcan
occurasanearlymanifestationofAIDSeveninpatientswith
apreservedimmunestatus.Diagnosticsuspicionshouldlead
toanearlybiopsyduetotherapidcourseofthistypeof
lym-phoma,withprompttreatmentperformedbyahematologist
548 MascarenhasJGetal.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
References
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