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Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired X-linked, clonal hematopoietic stem cell disease. Patients with PNH may complain of vague symptomatology that contributes to the challenge of its diagnosis. This is especially true in the clinical context of a coinciding hematologic disorder....
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9946272/ https://www.ncbi.nlm.nih.gov/pubmed/36843778 http://dx.doi.org/10.7759/cureus.34093 |
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author | Rayas, Jose Hassan, Mariam Hock, Rivers A Nguyen, Bryan Prakash, Swathi Rojas Murguia, Adrian Vahora, Ilma Corral, Javier Padilla, Osvaldo Dihowm, Fatma |
author_facet | Rayas, Jose Hassan, Mariam Hock, Rivers A Nguyen, Bryan Prakash, Swathi Rojas Murguia, Adrian Vahora, Ilma Corral, Javier Padilla, Osvaldo Dihowm, Fatma |
author_sort | Rayas, Jose |
collection | PubMed |
description | Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired X-linked, clonal hematopoietic stem cell disease. Patients with PNH may complain of vague symptomatology that contributes to the challenge of its diagnosis. This is especially true in the clinical context of a coinciding hematologic disorder. Aplastic anemia (AA) is an additional immune-mediated illness that results in the destruction of hematopoietic precursors and pancytopenia. The authors encourage screening for PNH clones in patients initially diagnosed with AA, treating underlying hematologic disease to prevent clonal expansion, and further research to investigate the effectiveness of eculizumab in an unusual “classical” PNH secondary to AA with hypercellular bone marrow. |
format | Online Article Text |
id | pubmed-9946272 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-99462722023-02-23 Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria Rayas, Jose Hassan, Mariam Hock, Rivers A Nguyen, Bryan Prakash, Swathi Rojas Murguia, Adrian Vahora, Ilma Corral, Javier Padilla, Osvaldo Dihowm, Fatma Cureus Internal Medicine Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired X-linked, clonal hematopoietic stem cell disease. Patients with PNH may complain of vague symptomatology that contributes to the challenge of its diagnosis. This is especially true in the clinical context of a coinciding hematologic disorder. Aplastic anemia (AA) is an additional immune-mediated illness that results in the destruction of hematopoietic precursors and pancytopenia. The authors encourage screening for PNH clones in patients initially diagnosed with AA, treating underlying hematologic disease to prevent clonal expansion, and further research to investigate the effectiveness of eculizumab in an unusual “classical” PNH secondary to AA with hypercellular bone marrow. Cureus 2023-01-23 /pmc/articles/PMC9946272/ /pubmed/36843778 http://dx.doi.org/10.7759/cureus.34093 Text en Copyright © 2023, Rayas et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Internal Medicine Rayas, Jose Hassan, Mariam Hock, Rivers A Nguyen, Bryan Prakash, Swathi Rojas Murguia, Adrian Vahora, Ilma Corral, Javier Padilla, Osvaldo Dihowm, Fatma Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria |
title | Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria |
title_full | Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria |
title_fullStr | Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria |
title_full_unstemmed | Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria |
title_short | Attack of the Clones: A Patient With Untreated Aplastic Anemia Presenting With Classical Paroxysmal Nocturnal Hemoglobinuria |
title_sort | attack of the clones: a patient with untreated aplastic anemia presenting with classical paroxysmal nocturnal hemoglobinuria |
topic | Internal Medicine |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9946272/ https://www.ncbi.nlm.nih.gov/pubmed/36843778 http://dx.doi.org/10.7759/cureus.34093 |
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