eculizumab in the management of paroxysmal nocturnal hemoglobinuria: patient selection and special considerations

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ID: 129527
2016
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Abstract
Fatimah Al-Ani,1 Ian Chin-Yee,1 Alejandro Lazo-Langner1,2 1Department of Medicine, Division of Hematology, 2Department of Epidemiology and Biostatistics, Western University, London, ON, Canada Abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal disorder resulting from somatic mutation in the PIG-A gene leading to a deficiency of the membrane-anchoring molecule glycosylphosphatidylinositol. The lack of expression of two glycosylphosphatidylinositol-anchored proteins involved in the regulation of the complement system renders PNH erythrocytes susceptible to complement-mediated lysis. Clinical manifestations include thromboembolic disease, chronic kidney injury, pulmonary hypertension, smooth muscle dysfunction, and chronic hemolysis. Until recently, treatment was mainly supportive with most patients suffering from significant morbidity and shortened survival compared to age-matched controls. The development of eculizumab, a humanized monoclonal antibody directed against the terminal complement protein C5, has resulted in dramatic improvements of survival and reduction in complications. In this paper, we review some special considerations pertaining to the use of eculizumab for PNH. Keywords: somatic mutation, GPI, survival, hemolysis, MDS, anemia
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f2016therapeuticseculizumab Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors ;Al-Ani F;Chin-Yee I;Lazo-Langner A
Journal liver transplantation : official publication of the american association for the study of liver diseases and the international liver transplantation society
Year 2016
DOI
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URL
https://www.dovepress.com/eculizumab-in-the-management-of-paroxysmal-nocturnal-hemoglobinuria-pa-peer-reviewed-article-TCRM
Keywords
paroxysmal nocturnal hemoglobinuria

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