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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....

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Autores principales: Rayas, Jose, Hassan, Mariam, Hock, Rivers A, Nguyen, Bryan, Prakash, Swathi, Rojas Murguia, Adrian, Vahora, Ilma, Corral, Javier, Padilla, Osvaldo, Dihowm, Fatma
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
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.
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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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