Profound morphological changes in the erythrocytes and fibrin networks of patients with hemochromatosis or with hyperferritinemia, and their normalization by iron chelators and other agents

dc.contributor.authorPretorius, Etheresia
dc.contributor.authorBester, Janette
dc.contributor.authorVermeulen, Natasha
dc.contributor.authorLipinski, Boguslaw
dc.contributor.authorGericke, George Sebastian
dc.contributor.authorKell, Douglas B.
dc.contributor.emailresia.pretorius@up.ac.zaen_ZA
dc.date.accessioned2015-05-28T09:14:35Z
dc.date.available2015-05-28T09:14:35Z
dc.date.issued2014-01-09
dc.description.abstractIt is well-known that individuals with increased iron levels are more prone to thrombotic diseases, mainly due to the presence of unliganded iron, and thereby the increased production of hydroxyl radicals. It is also known that erythrocytes (RBCs) may play an important role during thrombotic events. Therefore the purpose of the current study was to assess whether RBCs had an altered morphology in individuals with hereditary hemochromatosis (HH), as well as some who displayed hyperferritinemia (HF). Using scanning electron microscopy, we also assessed means by which the RBC and fibrin morphology might be normalized. An important objective was to test the hypothesis that the altered RBC morphology was due to the presence of excess unliganded iron by removing it through chelation. Very striking differences were observed, in that the erythrocytes from HH and HF individuals were distorted and had a much greater axial ratio compared to that accompanying the discoid appearance seen in the normal samples. The response to thrombin, and the appearance of a platelet-rich plasma smear, were also markedly different. These differences could largely be reversed by the iron chelator desferal and to some degree by the iron chelator clioquinol, or by the free radical trapping agents salicylate or selenite (that may themselves also be iron chelators). These findings are consistent with the view that the aberrant morphology of the HH and HF erythrocytes is caused, at least in part, by unliganded (‘free’) iron, whether derived directly via raised ferritin levels or otherwise, and that lowering it or affecting the consequences of its action may be of therapeutic benefit. The findings also bear on the question of the extent to which accepting blood donations from HH individuals may be desirable or otherwise.en_ZA
dc.description.librarianam2015en_ZA
dc.description.sponsorshipThe National Research Foundation of South Africa (NRF)en_ZA
dc.description.urihttp://www.plosone.orgen_ZA
dc.identifier.citationPretorius E, Bester J, Vermeulen N, Lipinski B, Gericke GS & Kell, D (2014) Profound Morphological Changes in the Erythrocytes and Fibrin Networks of Patients with Hemochromatosis or with Hyperferritinemia, and Their Normalization by Iron Chelators and Other Agents. PLoS ONE 9(1): e85271. DOI: 10.1371/journal.pone.0085271en_ZA
dc.identifier.issn1932-6203
dc.identifier.other10.1371/journal.pone.0085271
dc.identifier.urihttp://hdl.handle.net/2263/45323
dc.language.isoenen_ZA
dc.publisherPublic Library of Scienceen_ZA
dc.rights© 2014 Pretorius et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License.en_ZA
dc.subjectErythrocytesen_ZA
dc.subjectFibrin networksen_ZA
dc.subjectPatientsen_ZA
dc.subjectIron chelatorsen_ZA
dc.subjectThrombotic diseasesen_ZA
dc.subjectHereditary hemochromatosis (HH)en_ZA
dc.subjectHyperferritinemia (HF)en_ZA
dc.titleProfound morphological changes in the erythrocytes and fibrin networks of patients with hemochromatosis or with hyperferritinemia, and their normalization by iron chelators and other agentsen_ZA
dc.typeArticleen_ZA

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