Cargando…
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...
Autores principales: | , , , , , , |
---|---|
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 |
_version_ | 1783472608576012288 |
---|---|
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. |
format | Online Article Text |
id | pubmed-6873095 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT eisamahmouds paroxysmalnocturnalhemoglobinuriawithglucose6phosphatedehydrogenasedeficiencyacasereportandreviewoftheliterature AT mohamedshehabf paroxysmalnocturnalhemoglobinuriawithglucose6phosphatedehydrogenasedeficiencyacasereportandreviewoftheliterature AT ibrahimfiryal paroxysmalnocturnalhemoglobinuriawithglucose6phosphatedehydrogenasedeficiencyacasereportandreviewoftheliterature AT shariffkhalid paroxysmalnocturnalhemoglobinuriawithglucose6phosphatedehydrogenasedeficiencyacasereportandreviewoftheliterature AT sadiknagham paroxysmalnocturnalhemoglobinuriawithglucose6phosphatedehydrogenasedeficiencyacasereportandreviewoftheliterature AT nashwanabdulqadir paroxysmalnocturnalhemoglobinuriawithglucose6phosphatedehydrogenasedeficiencyacasereportandreviewoftheliterature AT yassinmohameda paroxysmalnocturnalhemoglobinuriawithglucose6phosphatedehydrogenasedeficiencyacasereportandreviewoftheliterature |