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Nasopharyngeal Burkitt lymphoma as an early AIDS manifestation

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BrazJOtorhinolaryngol.2014;80(6):546---548

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

www.bjorl.org

CASE

REPORT

Nasopharyngeal

Burkitt

lymphoma

as

an

early

AIDS

manifestation

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).

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

(2)

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

(3)

548 MascarenhasJGetal.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

1.BlumKA,LozanskiG,ByrdJC.AdultBurkittleukemiaand lym-phoma.Blood.2004;104:3009---20.

2.MolyneuxEM,RochfordR,GriffinB,NewtonR,JacksonG,Menon G,etal.Burkitt’slymphoma.Lancet.2012;379:1234---44.

3.Araújo I, Foss HD, Bittencourt A, Hummel M, Demel G, Mendonc¸aN,etal.ExpressionofEpstein---Barrvirus-gene prod-uctsinBurkitt’slymphomainNortheastBrazil.Blood.1996;87: 5279---86.

Imagem

Figure 1 Computed tomography of paranasal sinuses. (A) Coronal view, bone window and (B) axial view, soft tissue window, with a soft tissue density lesion in the nasopharynx, nasal cavity, and sinuses; magnetic resonance imaging of paranasal sinuses, (C) a

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