反复短程化疗长期存活非霍奇金淋巴瘤一例报导

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1、IntroductionBackgroundPureredcellaplasia(PRCA)describesaconditioninwhichRBCprecursorsinbonemarrowarenearlyabsent,whilemegakaryocytesandWBCprecursorsareusuallypresentatnormallevels.In1922,Kaznelsonrecognizedthatthisconditionwasadifferententitythanaplasticanemia.Pur

2、eredcellaplasiaexistsinseveralforms,andthemostcommonformisanacuteself-limitedcondition.Acquiredpureredcellaplasiaisoftenchronicandisassociatedwithunderlyingdisorderssuchasthymomasandautoimmunediseases.AcongenitalformofpureredcellaplasiawasinitiallydescribedbyJosep

3、hin1936andbyDiamond-Blackfanin1938.Congenitalpureredcellaplasiaisalifelongdisorder,anditisassociatedwithphysicalabnormalities.Bothacquiredandcongenitalpureredcellaplasiaareoccasionallyrefractorytotherapy.RecentresearchBecausePRCAisoneoftheautoimmunediseasesobserve

4、dinpatientswithlymphoma,Hirokawaetalattemptedtodiscerntherelationshipbetweenthe2conditions,1assessingthediseasecharacteristicsin8patientswhohadbothofthesedisorders.HalfofthepatientswerefoundtohaveB-celllymphoma,andtherestofthemhadtheT-celltype.In4patients,PRCAandl

5、ymphomadevelopedsimultaneously;in3ofthem,PRCAdevelopedaftertheappearanceoflymphoma(with2ofthesepatientsdevelopinganemiawhiletheirlymphomawasinremission);andin1patient,PRCAdevelopedfirst.Chemotherapyand/orimmunosuppressivetherapyprovedtobeeffectiveagainstanemiain7p

6、atients,withPRCAremainingindurableremissionin4oftheseindividualswithouttheuseofmaintenanceimmunosuppressivetherapy.Basedontheirresults,theauthorssuggestedthatlymphoma-associatedPRCAislinkedtoaheterogeneousmechanism.PathophysiologyErythroidprecursorsinbonemarroware

7、theprimarytargetsinpureredcellaplasia.Asaresult,patientscandevelopanormoblasticnormochromicanemiaandavirtualabsenceofreticulocytes.Injurytostemcellsinuteroisbelievedtobetheetiologyofapproximately90%ofcasesofcongenitalpureredcellaplasia(ie,Diamond-Blackfansyndrome)

8、.2Thistheoryisbasedonevidencethatcongenitalpureredcellaplasiaisfrequentlyassociatedwithrandomphysicalabnormalities,whileitisrarelyfamilialorassociatedwi