Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series.
Anemia, Aplastic
Flow Cytometry
Granulocytes
Hemoglobinuria, Paroxysmal
Oman
Quantification
Journal
Oman medical journal
ISSN: 1999-768X
Titre abrégé: Oman Med J
Pays: Oman
ID NLM: 101526350
Informations de publication
Date de publication:
Jul 2022
Jul 2022
Historique:
received:
17
08
2020
accepted:
23
12
2020
entrez:
11
8
2022
pubmed:
12
8
2022
medline:
12
8
2022
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 35949713
doi: 10.5001/omj.2022.13
pii: OMJ-37-04-2000167
pmc: PMC9357272
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e405Informations de copyright
The OMJ is Published Bimonthly and Copyrighted 2022 by the OMSB.
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