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Por favor, use este identificador para citar o enlazar a este item: http://hdl.handle.net/10261/149512
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dc.contributor.authorSubías, Martaes_ES
dc.contributor.authorMartín Merinero, Héctores_ES
dc.contributor.authorLópez, Aliciaes_ES
dc.contributor.authorAnter, Jaouades_ES
dc.contributor.authorPinto, Sheilaes_ES
dc.contributor.authorRodríguez de Córdoba, Santiagoes_ES
dc.date.accessioned2017-05-09T12:03:11Z-
dc.date.available2017-05-09T12:03:11Z-
dc.date.issued2017-02-
dc.identifier.citationImmunobiology 222 (2)363–371 (2017)es_ES
dc.identifier.issn0171-2985-
dc.identifier.urihttp://hdl.handle.net/10261/149512-
dc.description35 p.-3 fig.-4 tab. Subías, Marta et al.es_ES
dc.description.abstractParoxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia characterized by complement-mediated intravascular hemolysis that is effectively treated with eculizumab. However, treatment responses are reported heterogeneous with some patients presenting residual hemolysis and requiring RBC transfusions. Recent reports have shown that both extravascular hemolysis and incomplete C5 blockade can explain these suboptimal hematological responses. Here we have tested our eculizumab-treated PNH patients (n=12) for signs of hemolysis and assessed complement biomarkers. Patients were also genotyped for complement receptor 1 (CR1, CD35) and C5 polymorphisms and evaluated for free eculizumab in plasma. We report that 10 patients (83%) present parameters suggesting persistent hemolysis, although they did not require additional transfusions. Seven of them (58%) become direct Coombs-test positive as a consequence of treatment, including all patients carrying the low-expression CR1-L allele. CH50 and sC5b-9 assays demonstrate that the persistent low-level hemolysis identified in our treated patients is not a consequence of incomplete C5 blockade, supporting that this hemolysis, as has been suggested previously, results from the extravascular removal of C3 opsonized PNH erythrocytes. We also show that continuous alternative pathway activation in eculizumab-treated individuals carrying the CR1-L allele results in abnormally decreased levels of C3 in plasma that could, potentially, increase their susceptibility to bacterial infections. Finally, we encourage a routine evaluation of free eculizumab levels and terminal pathway activity to personalize eculizumab administration.es_ES
dc.description.sponsorshipS.RdeC is supported by the Spanish “Ministerio de Economía y Competitividad” (SAF2011-26583) and the Autonomous Region of Madrid (S2010/BMD-2316).es_ES
dc.language.isoenges_ES
dc.publisherElsevieres_ES
dc.relation.isversionofPostprintes_ES
dc.rightsopenAccessen_EN
dc.subjectPNHes_ES
dc.subjectComplementes_ES
dc.subjectExtravascular hemolysises_ES
dc.subjectComplement receptor 1es_ES
dc.subjectEculizumabes_ES
dc.titleExtravascular hemolysis and complement consumption in Paroxysmal Nocturnal Hemoglobinuria patients undergoing eculizumab treatmentes_ES
dc.title.alternativeHemolysis in eculizumab-treated PNH patientses_ES
dc.typeartículoes_ES
dc.identifier.doi10.1016/j.imbio.2016.09.002-
dc.description.peerreviewedPeer reviewedes_ES
dc.relation.publisherversionhttps://doi.org/10.1016/j.imbio.2016.09.002es_ES
dc.identifier.e-issn1878-3279-
dc.embargo.terms2018-02-28es_ES
dc.rights.licensehttp://creativecommons.org/licenses/by-nc-nd/4.0/-
dc.contributor.funderMinisterio de Economía y Competitividad (España)es_ES
dc.contributor.funderComunidad de Madrides_ES
dc.relation.csicSíes_ES
oprm.item.hasRevisionno ko 0 false*
dc.identifier.funderhttp://dx.doi.org/10.13039/501100003329es_ES
dc.identifier.funderhttp://dx.doi.org/10.13039/100012818es_ES
dc.type.coarhttp://purl.org/coar/resource_type/c_6501es_ES
item.openairetypeartículo-
item.grantfulltextopen-
item.cerifentitytypePublications-
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.fulltextWith Fulltext-
item.languageiso639-1en-
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