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ReportDOI:
FatalMicroangiopathicHemolyticAnemiaDueto
SézarySyndrome
,,LoriSoma2,AndreiShustov3,
,UniversityofWashington,Seattle,,Departmentof
Pathology,UniversityofWashington,Seattle,,DepartmentofMedicine,Universityof
Washington,Seattle,,DepartmentofMedicine,UniversityofWashington,Seattle,USA
Correspondingauthor:,******@
Abstract
Sézarysyndrome(SS)isaformofcutaneousT-celllymphoma(CTCL),demonstratingleukemicinvolvement
ofmalignantT--inducedanemia,
normocyticanemiasecondarytobonemarrowinfiltration,,
initiallydemonstratingwidespreadmorbilliformeruption,whopresentedwithmalignancy-related
microangiopathichemolyticanemia(MAHA).OurfindingsrepresentanovelpresentationofSSthatwill
informthedifferentialdiagnosisandtreatmentoffutureSSpatientspresentingwithanemiaand
thrombocytopenia.
Categories:Dermatology,Oncology
Keywords:sézarysyndrome,lymphoma,anemia,thrombocytopenia,hemolysis
Introduction
Sézarysyndrome(SS)isasubtypeofcutaneousT-celllymphoma(CTCL)primarilyoriginatingfrommatured
T-lymphocytesthatarepresentintheskinandblood[1].SSaffectsolderpatientsprimarilyovertheageof
60,ismorecommoninmales,andhasanincidenceofabout3:1,000,000intheUS[2].Whileanemiaand
thrombocytopeniahavepreviouslybeenreportedinpatientswithSSsecondarytobonemarrowinfiltration,
autoimmunehemolysis,ortreatmentwithparticularantineoplasticagents,therehavenotbeenprevious
reportsofamicroangiopathichemolyticanemia(MAHA)secondarytoprimarySS[3].
CasePresentation
A77-year-oldfemalewasdiagnosedwithSSonthebasisofapruritic,subtlebutwidespreadmorbilliform
-cell
populationofCD4+/CD5+T-cells,withaCD4/,
withaSézarycountof6,600cells/µ
Reviewbegan04/15/%usedoncedaily,
Reviewended06/04/2021wasfollowedcloselywithphysicalexamsandlaboratorystudiesandnotedoverthenextseveralyearsto
Published06/06/2021haveaslowincreaseincirculatingSézarycountandelevationoftheCD4/
©Copyright2021concomitantdecreaseintheoverallpercentofnaturalkiller(NK)cellsandmildthrombocytopeniawithout
.
articledistributedunderthetermsofthe
CreativeCommonsAttributionLicense
Threeyearsafterherinitialpresentation,thepatientpresentedwithfatigue,shortnessofbreath,andlower
CC-.,whichpermitsunrestricted
use,distribution,
medium,providedtheoriginalauthorandhematocritof12%,/µL,ameancorpuscularvolume(MCV)of
,aredcelldistributionwidth(RDW),aplateletcountof138,000cells/µL,anabsolute
/µL,alactatedehydrogenase(LDH)at262units/L,µmol/L,
andahaptoglobinat35mg/(DAT)(Figures
1A,1B)atthetimerevealedmarkedinvolvementbyherT-cellneoplasmwithover70%Sézarycellsby
morphology;%T-cellswithslightlydecreased
expressionofCD2,uniformlypositiveexpressionofCD4,slightlyincreasedexpressionofCD5,variably
decreasedexpressionofCD7,(Figure2).
Withthepatient’selevatedLDH,decreasedhaptoglobin,schistocytes,negativeCoombs,nearupperlimit
reticulocytecount,andsevereanemia,shewasdiagnosedwithanon-autoimmune,MAHAinthesettingof
SS.
Howtocitethisarticle
RobertsonJC,JafryMA,SomaL,etal.(June06,2021)FatalMicroangiopathicHemolyticAnemiaDuetoSé(6):
FIGURE1:Bonemarrowbiopsyshowsinfiltrationwith70%Sézarycells
bymorphology.(A)x10magnificationand(B)x40magnification.
FIGURE2:Peripheralbloodsmearshowsthepresenceofschistocytes,
indicativeofamicroangiopathic-hemolyticprocess.
months,herhemoglobinandplateletlevelshadstabilizedwithoutfurthertransfusion,andsheremained
presentation.
Discussion
SShasbeenassociatedwithhemophagocyticsyndrome-inducedanemia,normocyticanemiasecondaryto
bonemarrowinfiltration,andpancytopenia[4-6].Thromboticthrombocytopenicpurpura-hemolyticuremic
syndrome(TTP-HUS)hasbeenobservedasdose-dependenttoxicityofpentostatintreatmentforSS[7].
However,nosingle,commonformofanemiahasbeenfoundtobepresentinallSSpatients.
Otherdiagnosesconsideredinthedifferentialdiagnosisforourpatientincludedanemiasecondaryto
advanceddisease,thepatienthadanelevatedreticulocytecount,demonstratingcapacityforbonemarrow
patientswithSS[8].Inthiscase,thepatient’,thepresenceofschistocytesonthe
(6):
(HUS)and
thromboticthrombocytopenicpurpura(TTP)weregivenconsiderationinthiscontext,butwithnormalrenal
functionandnegativefeverorneurologicsymptoms,bothdiagnoseswereessentiallyexcluded.
Malignancy-associatedMAHAhasbeenpreviouslyreported[9-11].Themostcommonmalignancies
associatedincludecolon,gastric,prostate,lung,andcarcinomaofunknownprimary(CUP).Inmanyofthese
cases,MAHAwasoneofthefirstpresentationsofmalignancy,andextensiveworkupforTTP,HUS,and
disseminatedintravascularcoagulation(DIC)
fortheassociationofMAHAandmalignancyincludearteriolarorcapillaryswellingsecondarytoendothelial
damageofthemarrowvasculaturefromtheinvasionofthemalignancy,leadingtotheproductionoflarge
VonWillebrandFactor(VWF)multimersandsubsequentlycreatingamicroangiopathichemolyticprocess
similartoTTP[9,10,12].Similarly,ithasbeenproposedthattumoremboliorintraluminalfibrinthrombi
secondarytohypercoagulationmayleadtodirectbloodvesseldamage,creatinganenvironmentforRBC
fragmentation[9,10,12,13].Inthecaseofgastriccancers,theproductionofendothelium-damagingmucin
hasbeenproposedasapotentialmechanismfordirectbloodvesseldamage[14].
Theprimarytreatmentformalignancy-associatedMAHAisthetreatmentoftheunderlyingmalignancy
[9,10].Inmanycases,controloftheprimarymalignancyhassubsequentlycontrolledthehemolyticanemia
andstabilizedbloodcounts[15].TreatmentsutilizedforTTP-HUS,suchasrecombinantADAMTS13and
plasmaexchange,havebeenshowntodemonstrateefficacyintreatingmalignancy-associatedMAHA[16].
Theaveragesurvivalofmalignancy-relatedMAHAisfourmonthsforpatientsreceivinganti-cancertherapy
(includingchemotherapyorcancersurgery),-cancertherapy,
withassociatedsubgroupvariationbytypeofmalignancy[15].
Conclusions
Inconclusion,wepresentacaseofapatientwithSSpresentingwithmalignancy-
importantfortreatinghematologists/oncologistsanddermatologistswhoencounterpatientswithanemia
andthrombocytopeniainthesettingofSStonotonlyruleoutHUS,TTP,andanemiasecondarytodirect
infiltrationofthebonemarrow,
malignancy-associatedMAHAprimarilyincludescontroloftheunderlyingmalignancy;furtherinvestigation
isrequiredtodetermineefficacioustreatmentmodalitiesspecifictomalignancy-associatedMAHA.
AdditionalInformation
Disclosures
Humansubjects::In
compliancewiththeICMJEuniformdisclosureform,allauthorsdeclarethefollowing:Payment/services
info:Allauthorshavedeclaredthatnofinancialsupportwasreceivedfromanyorganizationforthe
:Allauthorshavedeclaredthattheyhavenofinancial
relationshipsatpresentorwithinthepreviousthreeyearswithanyorganizationsthatmighthavean
:Allauthorshavedeclaredthattherearenoother
relationshipsoractivitiesthatcouldappeartohaveinfluencedthesubmittedwork.
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