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Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series
We aimed to estimate the nature and prevalence of paroxysmal nocturnal hemoglobinuria (PNH) among Omani patients. We performed a retrospective review of all patients who were tested for PNH by flow cytometry at the Sultan Qaboos University Hospital, Muscat, between 2012 and 2019. Manifestations, tre...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
OMJ
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9357272/ https://www.ncbi.nlm.nih.gov/pubmed/35949713 http://dx.doi.org/10.5001/omj.2022.13 |
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author | Al-Riyami, Arwa Z. Al-Kindi, Yahya Al-Qassabi, Jamal Al-Mamari, Sahimah Fawaz, Naglaa Al-Khabori, Murtadha Al-Huneini, Mohammed Al Kindi, Salam |
author_facet | Al-Riyami, Arwa Z. Al-Kindi, Yahya Al-Qassabi, Jamal Al-Mamari, Sahimah Fawaz, Naglaa Al-Khabori, Murtadha Al-Huneini, Mohammed Al Kindi, Salam |
author_sort | Al-Riyami, Arwa Z. |
collection | PubMed |
description | We aimed to estimate the nature and prevalence of paroxysmal nocturnal hemoglobinuria (PNH) among Omani patients. We performed a retrospective review of all patients who were tested for PNH by flow cytometry at the Sultan Qaboos University Hospital, Muscat, between 2012 and 2019. Manifestations, treatment modalities, and outcomes were assessed. A total of 10 patients were diagnosed or were on follow-up for PNH (median age 22.5 years). Clinical manifestations included fatigue (80%) and anemia (70%). Six patients had classical PNH with hemolysis, three had PNH in the context of aplastic anemia, and one patient had subclinical PNH. The median total clone size (type II + III) for neutrophils was 95.5 (range: 1.5–97) (FLAER/CD24) and for monocytes was 91.6 (range = 0.04–99) (FLAER/CD14). Four patients had clone sizes > 50% at the time of diagnosis. The median follow-up period of the patients was 62 months (range = 8–204 months). One patient suffered thrombosis. Three patients were on immunosuppressant agents, five were initiated on eculizumab, and four had a bone marrow transplant. No deaths were reported in the cohort. The estimated average incidence of PNH among Omani patients was 1.5 per 5 000 000. PNH is rare in the Omani population. The predominant presentation is hemolytic anemia. |
format | Online Article Text |
id | pubmed-9357272 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | OMJ |
record_format | MEDLINE/PubMed |
spelling | pubmed-93572722022-08-09 Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series Al-Riyami, Arwa Z. Al-Kindi, Yahya Al-Qassabi, Jamal Al-Mamari, Sahimah Fawaz, Naglaa Al-Khabori, Murtadha Al-Huneini, Mohammed Al Kindi, Salam Oman Med J Case Reports We aimed to estimate the nature and prevalence of paroxysmal nocturnal hemoglobinuria (PNH) among Omani patients. We performed a retrospective review of all patients who were tested for PNH by flow cytometry at the Sultan Qaboos University Hospital, Muscat, between 2012 and 2019. Manifestations, treatment modalities, and outcomes were assessed. A total of 10 patients were diagnosed or were on follow-up for PNH (median age 22.5 years). Clinical manifestations included fatigue (80%) and anemia (70%). Six patients had classical PNH with hemolysis, three had PNH in the context of aplastic anemia, and one patient had subclinical PNH. The median total clone size (type II + III) for neutrophils was 95.5 (range: 1.5–97) (FLAER/CD24) and for monocytes was 91.6 (range = 0.04–99) (FLAER/CD14). Four patients had clone sizes > 50% at the time of diagnosis. The median follow-up period of the patients was 62 months (range = 8–204 months). One patient suffered thrombosis. Three patients were on immunosuppressant agents, five were initiated on eculizumab, and four had a bone marrow transplant. No deaths were reported in the cohort. The estimated average incidence of PNH among Omani patients was 1.5 per 5 000 000. PNH is rare in the Omani population. The predominant presentation is hemolytic anemia. OMJ 2022-07-31 /pmc/articles/PMC9357272/ /pubmed/35949713 http://dx.doi.org/10.5001/omj.2022.13 Text en The OMJ is Published Bimonthly and Copyrighted 2022 by the OMSB. https://creativecommons.org/licenses/by-nc/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial (CC BY-NC) 4.0 License. http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) |
spellingShingle | Case Reports Al-Riyami, Arwa Z. Al-Kindi, Yahya Al-Qassabi, Jamal Al-Mamari, Sahimah Fawaz, Naglaa Al-Khabori, Murtadha Al-Huneini, Mohammed Al Kindi, Salam Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series |
title | Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series |
title_full | Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series |
title_fullStr | Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series |
title_full_unstemmed | Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series |
title_short | Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series |
title_sort | clinicopathological profile of paroxysmal nocturnal hemoglobinuria among omani patients: a case series |
topic | Case Reports |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9357272/ https://www.ncbi.nlm.nih.gov/pubmed/35949713 http://dx.doi.org/10.5001/omj.2022.13 |
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