Evidence that Prefibrotic Myelofibrosis Is Aligned along a Clinical and Biological Continuum Featuring Primary Myelofibrosis 英文参考文献.docVIP
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Evidence that Prefibrotic Myelofibrosis Is Aligned along a Clinical and Biological Continuum Featuring Primary Myelofibrosis 英文参考文献
EvidencethatPrefibroticMyelofibrosisIsAlignedalonga
ClinicalandBiologicalContinuumFeaturingPrimary
Myelofibrosis
GiovanniBarosi1*,VittorioRosti1,ElisaBonetti1,RitaCampanelli1,AdrianaCarolei1,PaoloCatarsi1,
AntoninaM.Isgro`1,LetiziaLupo1,MargheritaMassa2,ValentinaPoletto1,GianlucaViarengo3,
LauraVillani1,UmbertoMagrini1
1LaboratoryofClinicalEpidemiologyandCentrefortheStudyofMyelofibrosis,IRCCSPoliclinicoS.MatteoFoundation,Pavia,Italy,2BiotechnologyResearchArea,IRCCS
PoliclinicoS.MatteoFoundation,Pavia,Italy,3UnitofClinicalImmunology,Immunohematology,andTransfusionService,IRCCSPoliclinicoS.MatteoFoundation,Pavia,
Italy
Abstract
Purpose: In the WHO diagnostic classification, prefibrotic myelofibrosis (pre-MF) is included in the category of primary
myelofibrosis(PMF).However,strongevidenceforthispositionislacking.
PatientsandMethods:Weinvestigatedwhetherpre-MFmaybealignedalongaclinicalandbiologicalcontinuumin683
consecutivepatientswhoreceivedaWHOdiagnosisofPMF.
Results: As compared with PMF-fibrotic type, pre-MF (132 cases) showed female dominance, younger age, higher
hemoglobin,higherplateletcount,lowerwhitebloodcellcount,smallerspleenindexandhigherincidenceofsplanchnic
veinthrombosis.Femaletomaleratioandhemoglobinsteadilydecreased,whileageincreasedfrompre-MFtoPMF-fibrotic
typewithearlyandtoadvancedbonemarrow(BM)fibrosis.Likely,circulatingCD34+cells,LDHlevels,andfrequencyof
chromosomalabnormalitiesincreased,whileCXCR4expressiononCD34+cellsandserumcholesteroldecreasedalongthe
continuumofBMfibrosis.MediansurvivaloftheentirecohortofPMFcaseswas21years.Ninety-eight,eighty-oneandfifty-
sixpercentofpatientswithpre-MF,PMF-fibrotictypewithearlyandwithadvancedBMfibrosis,respectively,werealiveat
10yearsfromdiagnosis.
Conclusion: Pre-MF is a presentation mode of PMF with a very indolent phenotype. The major consequences of this
contentionisanewclinicalvisionofPMF,andtheneedtoimproveprognosispredictionofthedisease.
Citation: Barosi G, Rosti V, Bonetti E, Campanelli R, Carolei A,
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