Rapidly evolving skin manifestations due to progressive thrombosis in a patient with paroxysmal nocturnal haemoglobinuria resolved with prompt initiation of eculizumab
SourceBritish Journal of Dermatology, 171, 4, (2014), pp. 908-910
Article / Letter to editor
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British Journal of Dermatology
SubjectRadboudumc 16: Vascular damage RIHS: Radboud Institute for Health Sciences; Radboudumc 5: Inflammatory diseases RIHS: Radboud Institute for Health Sciences
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disorder of the haematopoietic stem cell. PNH arises from mutations in the PIG-A gene encoding glycosylphosphatidylinositol (GPI) anchored proteins consequently resulting in deficiency of GPI-anchored proteins the cell membranes.1 This deficiency causes PNH red cells to be susceptible for complement induced intravascular hemolysis.2 However, PNH is also characterized by a high rate of thrombosis.3-4 This article is protected by copyright. All rights reserved.
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