SuccessfulTreatmentwithEculizumabin a Patientwith Pregnancy-AssociatedAtypicalHemolyticUremicSyndrome


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Duran M. N. , Beyazıt F. , Erbaş M. , Acar C. , Bakırdöğen S.

TJOD 2020, İstanbul, Türkiye, 4 - 06 Aralık 2020, ss.0-1

  • Basıldığı Şehir: İstanbul
  • Basıldığı Ülke: Türkiye
  • Sayfa Sayıları: ss.0-1

Özet

SuccessfulTreatmentwithEculizumabin a Patientwith  Pregnancy-AssociatedAtypicalHemolyticUremicSyndrome

Mehmet Nuri̇Duran1, Fatma Beyazit1, Mesut Erbaş2, Onur Özkavak1, Celal Acar3, Serkan Bakirdogen3

1Çanakkale OnsekizMart University, Departmentof ObstetricsandGynecology, Çanakkale

2Çanakkale OnsekizMart University, Departmentof AnesthesiologyandReanimation, Çanakkale

3Çanakkale OnsekizMart University, Departmentof InternalMedicine, Çanakkale

Pregnancyisaconditionthatpredisposeswomentoincreasedriskfordevelopingthromboticthrombocytopenicpurpura(TTP)andhemolyticuremicsyndrome(HUS),collectivelyreferredtoasthromboticmicroangiopathies(TMAs).ClinicalandlaboratoryfeaturesofTMAsareheterogeneous,andcommoncharacteristicsincludemicroangiopathichemolysis,thrombocytopeniaandthrombiinsmallvesselsleadingtoendorgandamage,primarilykidneyinvolvement.Althoughformanyyears,theclassificationofTMAshasappearedtobechallenging,theEuropeanStudyGroupforHemolyticUremicSyndrome(HUS)andrelatedsyndromesrecommendedarecentclassificationinwhichHUSbeclassifiedintothreeprimarytypes:(1)HUSduetoinfections,HUSrelatedtocomplementabnormalitiesorrelatedtofactorADAMTS13deficiency,knownasatypicalHUS(aHUS)andHUSofunknownetiologythattypicallyoccursinthecourseofsystemicdiseasesorphysiopathologicconditions,suchaspregnancy,aftertransplantationordrugingestion(knownassecondaryHUS).Pregnancy-associatedaHUS(P-aHUS)canbeconfusedwithpreeclampsiabecauseofsimilarclinicalmanifestationsduetoendothelial-injury-relatedtissuedamageoccurringinbothconditions..

INTRODUCTIONCASE

CONCLUSION

SuccessfulTreatmentwithEculizumabin a Patientwith

Pregnancy-AssociatedAtypicalHemolyticUremicSyndrome

EculizumabisahumanisedmonoclonalIgG2/4κantibodyproducedfrommurinemyelomacellsbyrecombinantDNAtechnologyandisproventobeeffectiveinbothparoxysmalnocturnalhemoglobinuria(PNH)andcomplement-relatedHUS.Itwasoriginallytestedforrheumatoidarthritiswithoutsuccess,andrecentlydemonstratedtobeeffectiveinpatientswithaHUS.MoreoveracumulatingevidencesuggeststhateculizumabissuperiortoplasmatherapyininducingremissioninpatientswithacuteaHUS.Thiscasedemonstratesthesignificanceofearlyinitiationofanti-complementtherapytopreventirreversiblerenaldamageandpossibledeathinpatientswithP-aHUS..

Figure1.GraphicpresentationoflaboratoryresultsofthepatientwhilereceivingplasmapheresisandEculizumabtreatment

Inthiscasereport,wepresenta35-year-oldgravida3,parity2womanat32weeksgestationwhounderwentcesareansectionduetofetaldistress.Thefirst6hoursafterLSCSwereuneventful,but6hoursafteroperation,herurineoutputbegantodecline,andshealsohadanemia,thrombocytopeniaandincreasedserumbloodureanitrogenandcreatininelevels.Atpost-operative24hours,shehadaseizureattack.Duetolaboratoryfindingsandhercurrentclinicalpresentation,adiagnosisofaHUSwasperformed.Shewastransferredtotheintensivecareunit(ICU)forfurthermanagement.Urgenttreatmentwithplasmapheresiswascommenced.Althoughslightimprovementwasobservedinthepatient’sclinicalstatusandlaboratoryvalues,eculizumabtreatmentwasinitialized.Thepatientreceivedeculizumabtreatmentonpostoperativeday12.Figure1presentsagraphicpresentationofthelaboratoryresultsofthepatientwhilereceivingplasmapheresisandeculizumabtreatment.Afteratotalofonedoseofeculizumab,renalfunctionimproved,andthereforethehemodialysissessionswereceased.Fourweeksfollowingadmission,thepatientwasdischargedfromthehospitalingoodhealth,andthelaboratorystudiesrevealedprogressiveameliorationofrenaldysfunctionandhematologicalabnormalities.