Pathology Unknown Case: April 2016 - ohsu.edu · Pathology Unknown Case: April 2016 Kyle Muir MS4...
Transcript of Pathology Unknown Case: April 2016 - ohsu.edu · Pathology Unknown Case: April 2016 Kyle Muir MS4...
ClinicalHistoryA71yohealthymalepresentedwithprogressivefaAgueandwasfoundtobepancytopenic.BonemarrowbiopsywasconsistentwithmyelodysplasAcsyndrome.HereceivedaCMV-BMTandwasadmiNed5weekspost-transplantwithvomiAnganddiarrhea.
MicroscopicDescripAon
GastricandduodenalmucosawithepithelialapoptosisanddropoutwithoutsignificantinflammatorycellinfiltraAon.
Whatisthediagnosis?
A. IschemiaB. InfecAousgastroenteriAsC. GraX-versus-hostdiseaseD. Autoimmuneenteropathy
ClinicalQuesAon
• WhichofthefollowingeAologiescanmostcloselyresemblethepathologyofthiscondiAon?
a. CMVreacAvaAonb. AcutecoliAsc. C.DifficileinfecAond. Ischemicbowel
Answer
a. CMVreacHvaHonCMVinfecAonoftheGItractcanpresentwithawidevarietyofpathologicfeaturesincludingthetypicalGVHDfindingsofcryptdropoutandepithelialapoptosis.Therefore,itiscrucialtolookforinclusions(unfortunatelycanberareorevenabsentinthesecases)andperformappropriateIHCtesAngifthereisanydoubt.
OverallPathologicImpression
• FurtherIHCtesAngwasnegaAveforCMV,herpesandadenovirus.
• OverallpathologyconsistentwithadiagnosisofgraX-versus-hostdisease(GVHD).
AcuteGVHDClinicalStaging
ClinicalStage Skin Liver—Bilirubin,μmol/L(mg/dL) Gut
1 Rash<25%bodysurface 34–51(2–3) Diarrhea500–1000
mL/d
2 Rash25–50%bodysurface 51–103(3–6) Diarrhea1000–
1500mL/d
3 Generalizederythroderma 103–257(6–15) Diarrhea>1500mL/
d
4 DesquamaAonandbullae >257(>15) Ileus
OverallClinicalGrade SkinStage LiverStage GutStage
I 1–2 0 0
II 1–3 1 1
III 1–3 2–3 2–3
IV 2–4 2–4 2–4
• MostcommonlyaffectedorgansinacuteGVHDareskin,liverandGItract.• BelowistheclinicalgradingschemeforacuteGVHD
AcuteGVHDoftheGItract• DefiniHon:aNackbyengraXedhematopoieAccellsonhostGIAssuesinfirst
100dayspost-transplant• DiagnosAcCriteria(GradeI-IV)
– IncreasedcryptapoptosisprogressingtocryptdestrucAonandmucosalnecrosisinhighergrades
• PotenHalPiNalls– ConsiderinfecAonifpresenceofacuteorchronicinflammaAonor
neutrophilicabscesses/ulceraAon.AlsokeepinmindthepossibilityofadiminishedinflammatorycellresponseintheseimmunocompromisedpaAents.
– UseIHCtocheckforCMV/herpes– BevigilantasinfecAoncanoXencoexistwithGVHDandhasaprofound
impactonmanagement
Grade1=isolatedapoptoAcepithelialcells,withoutcryptlossGrade2=individualcryptlossGrade3=conAguousareaofmulAplecryptlossGrade4=extensivecryptdropoutwithdenudaAonofepithelium.
References• HeymerB.ClinicalanddiagnosAcpathologyofgraXversushostdisease.
SpringerVerlag,2002.• ShulmanHMetal.HistopathologicdiagnosisofchronicgraX-versus-host
disease:NaAonalInsAtutesofHealthConsensusDevelopmentProjectonCriteriaforClinicalTrialsinChronicGraX-versus-HostDisease:II.PathologyWorkingGroupReport.BiolBloodMarrowTransplant.2006Jan;12(1):31-47.
• Kambham,Neeraja.SurgicalPathologyCriteria.AcuteGra*versusHostDisease(GVHD)ofGastrointes6nalTract.StanfordSchoolofMedicine,9May2007.Web.15Feb.2016.<hNp://surgpathcriteria.stanford.edu/transplant/giacutegvhd/printable.html>.
• LernerKG,KaoGF,StorbR,etal.HistopathologyofgraX-vs-hostreacAon(GvHR)inhumanrecipientsofmarrowfromHL-A-matchedsiblingdonors.TransplantProc1974;6:367–371.
• ModernPathology(2011)24,117–125;doi:10.1038/modpathol.2010.163;publishedonline15October2010