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Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury

Paroxysmal nocturnal hemoglobinuria is a rare form of intravascular hemolysis caused by an acquired deficiency of complement regulatory glycoproteins. In our case, a 53-year-old male presented with fatigue, discoloration of urine, and reduced urine output. Preliminary investigations showed severe an...

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Autores principales: N, Mohith H, Pinto, Christopher J, Poornima, Jana, Rajput, Ajay K, Bagheri, Marziyeh, Patil, Basawantrao, Nizamuddin, Mohammad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9417682/
https://www.ncbi.nlm.nih.gov/pubmed/36046061
http://dx.doi.org/10.7759/cureus.28448
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author N, Mohith H
Pinto, Christopher J
Poornima, Jana
Rajput, Ajay K
Bagheri, Marziyeh
Patil, Basawantrao
Nizamuddin, Mohammad
author_facet N, Mohith H
Pinto, Christopher J
Poornima, Jana
Rajput, Ajay K
Bagheri, Marziyeh
Patil, Basawantrao
Nizamuddin, Mohammad
author_sort N, Mohith H
collection PubMed
description Paroxysmal nocturnal hemoglobinuria is a rare form of intravascular hemolysis caused by an acquired deficiency of complement regulatory glycoproteins. In our case, a 53-year-old male presented with fatigue, discoloration of urine, and reduced urine output. Preliminary investigations showed severe anemia (3.7 g/dl) and hyperkalemia (7.6 mmol/L) in the setting of acute kidney injury, requiring urgent dialysis. Four units of packed cell volumes were transfused for the correction of anaemia. Following initial stabilization, flow cytometry and a fluorescein-labeled proaerolysin (FLAER) study showed a total deficiency of CD59 in 95.92% of granulocytes and a 97.14% deficiency in monocytes. A bone marrow biopsy showed erythroblast hyperplasia confirming the diagnosis of classical paroxysmal nocturnal hemoglobinuria. The patient was treated with steroids, androgens, and iron supplementation and made a complete recovery with a near-total resolution of his acute kidney injury. This paper aims to review the clinical features and investigations in order to focus on acute kidney injury as an outcome of paroxysmal nocturnal hemoglobinuria.
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spelling pubmed-94176822022-08-30 Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury N, Mohith H Pinto, Christopher J Poornima, Jana Rajput, Ajay K Bagheri, Marziyeh Patil, Basawantrao Nizamuddin, Mohammad Cureus Emergency Medicine Paroxysmal nocturnal hemoglobinuria is a rare form of intravascular hemolysis caused by an acquired deficiency of complement regulatory glycoproteins. In our case, a 53-year-old male presented with fatigue, discoloration of urine, and reduced urine output. Preliminary investigations showed severe anemia (3.7 g/dl) and hyperkalemia (7.6 mmol/L) in the setting of acute kidney injury, requiring urgent dialysis. Four units of packed cell volumes were transfused for the correction of anaemia. Following initial stabilization, flow cytometry and a fluorescein-labeled proaerolysin (FLAER) study showed a total deficiency of CD59 in 95.92% of granulocytes and a 97.14% deficiency in monocytes. A bone marrow biopsy showed erythroblast hyperplasia confirming the diagnosis of classical paroxysmal nocturnal hemoglobinuria. The patient was treated with steroids, androgens, and iron supplementation and made a complete recovery with a near-total resolution of his acute kidney injury. This paper aims to review the clinical features and investigations in order to focus on acute kidney injury as an outcome of paroxysmal nocturnal hemoglobinuria. Cureus 2022-08-26 /pmc/articles/PMC9417682/ /pubmed/36046061 http://dx.doi.org/10.7759/cureus.28448 Text en Copyright © 2022, N 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 Emergency Medicine
N, Mohith H
Pinto, Christopher J
Poornima, Jana
Rajput, Ajay K
Bagheri, Marziyeh
Patil, Basawantrao
Nizamuddin, Mohammad
Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury
title Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury
title_full Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury
title_fullStr Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury
title_full_unstemmed Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury
title_short Classical Paroxysmal Nocturnal Hemoglobinuria Presenting With Severe Anemia and Pigmented Acute Kidney Injury
title_sort classical paroxysmal nocturnal hemoglobinuria presenting with severe anemia and pigmented acute kidney injury
topic Emergency Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9417682/
https://www.ncbi.nlm.nih.gov/pubmed/36046061
http://dx.doi.org/10.7759/cureus.28448
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