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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...

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Autores principales: Al-Riyami, Arwa Z., Al-Kindi, Yahya, Al-Qassabi, Jamal, Al-Mamari, Sahimah, Fawaz, Naglaa, Al-Khabori, Murtadha, Al-Huneini, Mohammed, Al Kindi, Salam
Formato: Online Artículo Texto
Lenguaje:English
Publicado: OMJ 2022
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.
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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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