Pseudo (Platelet-type) von Willebrand disease in pregnancy a case report 英文参考文献.docVIP

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Pseudo (Platelet-type) von Willebrand disease in pregnancy a case report 英文参考文献.doc

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Pseudo (Platelet-type) von Willebrand disease in pregnancy a case report 英文参考文献

Groveretal.BMCPregnancyandChildbirth2013,13:16 /1471-2393/13/16 CASE REPORT OpenAccess Pseudo(Platelet-type)vonWillebranddiseasein pregnancy:acasereport NeetuGrover1,VincentBoama2*andMunazzahRifatChou1 Abstract Background:Pseudo(platelet-type)-vonWillebranddiseaseisarareautosomaldominantbleedingdisordercaused byanabnormalfunctionoftheglycoproteinlbprotein;thereceptorforvonWillebrandfactor.Thisleadstoan increasedremovalofVWFmultimersfromthecirculationaswellasplateletsandthisresultsinableedingdiathesis. Worldwide,lessthan50patientsarereportedwithplatelettypevonWillebranddisease(PT-VWD). Casepresentation:WedescribethemanagementofplatelettypevonWillebranddiseaseinpregnancyofa26 yearoldCaucasianprimigravida.Theinitialdiagnosiswasmadeearlierfollowingasignificanthaemorrhagepost tonsillectomyseveralyearspriortopregnancy.Thepatientwasmanagedunderamultidisciplinaryteamwhich includedobstetricians,haematologists,anaesthetistsandneonatologists.Careplansweremadefortheante-natal, intra-partumandpost-partumperiodsinpartnershipwiththepatient.Thepatient’splateletcountlevelsdropped significantlyduringtheantenatalperiod.Thisnecessitatedtheactiveexclusionofothercausesofthrombocytopeniain pregnancy.Avaginaldeliverywasdesiredandplansweremadeforinductionoflabourat38weeksofgestationwith plateletcoverinviewoftheprogressivefalloftheplateletcount.Thepatienthoweverwentintospontaneouslabour onthedayofinduction.Shewastransfusedtwounitsofplateletsbeforedelivery.Shehadanunassistedvaginal deliveryofahealthybaby.Thesuccessfulantenatalcounsellinghasencouragedthediagnosisofthesameconditionin hermotherandsister.Wefoundthistobeaparticularlyinterestingcaseaswellaschallengingtomanageduetoits rarity.PsuedovonWillebranddiseaseinpregnancycanbeconfusedwithanumberofotherdifferentialdiagnoses, suchasgestationalthrombocutopenia,idiopathaticthrombocytopenia,thromboticthrombocytopenicpurpuraand pre-eclampsia;allneedconsiderationduringinvestigationseveninacasesuchasthiswherethediagnosisofplatelet typevonWillebrand