Transcription of Immunobiology - SAH
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Immunobiology - SAH
Immunobiology 217 (2012) 1080 1087 Contents lists available atSciVerse ScienceDirectImmunobiologyjournal nocturnal hemoglobinuria and other complement-mediatedhematological disordersAntonio M. Risitano Hematology, Department of Biochemistry and Medical Biotechnologies, Federico II University, Naples, Italyarticle infoArticle history:Received 3 July 2012 Received in revised form 18 July 2012 Accepted 18 July 2012 Keywords:Paroxysmal nocturnal hemoglobinuriaComplement alternative pathwayComplement component 3 Complement component 5 EculizumabTT30C3-targeted therapyabstractThe recent availability of eculizumab as the first complement inhibitor renewed the interest forcomplement-mediated damage in several human diseases. Paroxysmal nocturnal hemoglobinuria (PNH)may be considered the paradigm a disease caused by complement dysregulation specifically on erythro-cytes; in fact, PNH is a clonal, non-malignant, hematological disorder characterized by the expansion ofhematopoietic stem cells and progeny mature blood cells which are deficient in some surface proteins,including the two complement regulators CD55 and CD59.
A.M. Risitano / Immunobiology 217 (2012) 1080–1087 1081 Complement dysregulation in PNH PNHisarareandpuzzlinghematologicaldisordercharacterized
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