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Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature

In this study, we are describing a female patient with paroxysmal nocturnal hemoglobinuria (PNH) and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Both diseases are known to cause hemolytic anemia that mediates the hemolysis of RBCs through several mechanisms. In PNH the hemolysis is mediated...

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Autores principales: Eisa, Mahmoud S., Mohamed, Shehab F., Ibrahim, Firyal, Shariff, Khalid, Sadik, Nagham, Nashwan, Abdulqadir, Yassin, Mohamed A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6873095/
https://www.ncbi.nlm.nih.gov/pubmed/31762758
http://dx.doi.org/10.1159/000503817
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author Eisa, Mahmoud S.
Mohamed, Shehab F.
Ibrahim, Firyal
Shariff, Khalid
Sadik, Nagham
Nashwan, Abdulqadir
Yassin, Mohamed A.
author_facet Eisa, Mahmoud S.
Mohamed, Shehab F.
Ibrahim, Firyal
Shariff, Khalid
Sadik, Nagham
Nashwan, Abdulqadir
Yassin, Mohamed A.
author_sort Eisa, Mahmoud S.
collection PubMed
description In this study, we are describing a female patient with paroxysmal nocturnal hemoglobinuria (PNH) and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Both diseases are known to cause hemolytic anemia that mediates the hemolysis of RBCs through several mechanisms. In PNH the hemolysis is mediated through complement activation and oxidative stress. G6PD enzyme is crucial in preventing damage to cellular structures caused by oxygen-free radicles. In G6PD deficiency the hemolysis is mediated through the oxidative stress created by oxygen-free radicles. Since both diseases mediate hemolysis through the oxidative stress, we hypothesize that both conditions have facilitated an effect on each other and this will reflect on the response to treatment, and this response to treatment could vary based on whether the two mutations occurred in the same gene or in two different X chromosomes. Having diagnosed PNH, the management is very expensive and not all the patients can afford it, especially our patient who is a maid by occupation. So, the real challenge in our case is to monitor her in subsequent visits and to plan the treatment keeping in mind her financial status.
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spelling pubmed-68730952019-11-22 Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature Eisa, Mahmoud S. Mohamed, Shehab F. Ibrahim, Firyal Shariff, Khalid Sadik, Nagham Nashwan, Abdulqadir Yassin, Mohamed A. Case Rep Oncol Case Report In this study, we are describing a female patient with paroxysmal nocturnal hemoglobinuria (PNH) and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Both diseases are known to cause hemolytic anemia that mediates the hemolysis of RBCs through several mechanisms. In PNH the hemolysis is mediated through complement activation and oxidative stress. G6PD enzyme is crucial in preventing damage to cellular structures caused by oxygen-free radicles. In G6PD deficiency the hemolysis is mediated through the oxidative stress created by oxygen-free radicles. Since both diseases mediate hemolysis through the oxidative stress, we hypothesize that both conditions have facilitated an effect on each other and this will reflect on the response to treatment, and this response to treatment could vary based on whether the two mutations occurred in the same gene or in two different X chromosomes. Having diagnosed PNH, the management is very expensive and not all the patients can afford it, especially our patient who is a maid by occupation. So, the real challenge in our case is to monitor her in subsequent visits and to plan the treatment keeping in mind her financial status. S. Karger AG 2019-11-01 /pmc/articles/PMC6873095/ /pubmed/31762758 http://dx.doi.org/10.1159/000503817 Text en Copyright © 2019 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Eisa, Mahmoud S.
Mohamed, Shehab F.
Ibrahim, Firyal
Shariff, Khalid
Sadik, Nagham
Nashwan, Abdulqadir
Yassin, Mohamed A.
Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature
title Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature
title_full Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature
title_fullStr Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature
title_full_unstemmed Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature
title_short Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature
title_sort paroxysmal nocturnal hemoglobinuria with glucose-6-phosphate dehydrogenase deficiency: a case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6873095/
https://www.ncbi.nlm.nih.gov/pubmed/31762758
http://dx.doi.org/10.1159/000503817
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