Clinicopathological Profile of Paroxysmal Nocturnal Hemoglobinuria among Omani Patients: A Case Series.

Autor:	Al-Riyami AZ; Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman., Al-Kindi Y; College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman., Al-Qassabi J; Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman., Al-Mamari S; Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman., Fawaz N; Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman., Al-Khabori M; Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman., Al-Huneini M; Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman., Al Kindi S; Department of Hematology, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman.
Jazyk:	angličtina
Zdroj:	Oman medical journal [Oman Med J] 2022 Jul 31; Vol. 37 (4), pp. e405. Date of Electronic Publication: 2022 Jul 31 (Print Publication: 2022).
DOI:	10.5001/omj.2022.13
Abstrakt:	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. (The OMJ is Published Bimonthly and Copyrighted 2022 by the OMSB.)
Databáze:	MEDLINE
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