Flow cytometry screening for paroxysmal nocturnal hemoglobinuria: A single‐center experience in Saudi Arabia

AlGhasham, Nahlah; Abulkhair, Yasmeen; Khalil, Salem

doi:10.1002/cyto.b.21317

Cited by 9 publications

(2 citation statements)

References 23 publications

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“…A study on the Saudi population showed 4% detectable PNH clones in tested patients, albeit 73% of them presented with AA. 19 In our cohort, more than half of the diagnosed patients had classical PNH with manifestations of DAT-negative hemolytic anemia and large PNH clones (> 60% neutrophils and monocytes). One of these patients presented with hemolytic anemia, aplastic marrow, and a large PNH clone, suggesting an overlap presentation.…”

Section: Discussionmentioning

confidence: 71%

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

Al-Riyami¹,

Al-Kindi²,

Al-Qassabi³

et al. 2022

Oman Med J

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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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Section: Discussionmentioning

confidence: 71%

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

Al-Riyami¹,

Al-Kindi²,

Al-Qassabi³

et al. 2022

Oman Med J

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“…In agreement with former studies, results of the current report show that the PNH clone sizes on RBCs are significantly smaller than clones on WBCs with a p ‐value <0.0001 using a Mann–Whitney test. This difference may be due to previous blood transfusions or the increased rate of haemolysis within this cohort of patients [ 19 ]. There's also a significant correlation between PNH clone sizes on different cell lineages with a Spearman r of 0.7316 for PNH clones on granulocytes and monocytes, and a Spearman r of 0.7813 for PNH clones on RBCs and granulocytes.…”

Section: Discussionmentioning

confidence: 99%

Paroxysmal nocturnal haemoglobinuria, diagnosis and haematological findings, first report from Iran, model for developing countries

Jahangirpour

Vahedi²,

Baghdadi

et al. 2022

eJHaem

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Since paroxysmal nocturnal haemoglobinuria (PNH) was first described in 1881, the diagnosis and follow-up patients diagnosed with the illness has remained an area of concern, with several different techniques of varying sensitivity having been described in the literature for both the diagnosis and monitoring treatment of the disease. PNH is a rare and life-threatening disease that manifests symptoms of haemolytic anaemia.Hence, a quick and reliable technique for precise diagnosis would be crucial. PNH patients who have previously been diagnosed with aplastic anaemia or myelodysplastic syndrome carry small PNH clones and for more than a century traditional method with low sensitivity was used for such patients. In 2010, the International Clinical Cytometry Society described a highly sensitive method for detection and quantification of different types of PNH clones using multi-colour flow cytometry. In this method, a threecolour flow cytometer is essential to detect PNH affected cells amongst monocytes and granulocytes. This started a new era in the diagnosis of patients who carry small clones of PNH cells. Before this, flow cytometric analysis was used only for detection of PNH cells amongst erythrocytes. By using flow cytometry instruments with more light sources, the sensitivity of detection and quantification of PNH clones would be augmented. However, standardisation and crosstalk compensation would be the most concerning issue. For the first time in Iran, we set up and standardised multi-colour flow cytometry technique to detect PNH cells in erythrocytes and leukocytes at Payvand medical laboratory.

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Epidemiology of Budd–Chiari Syndrome

Riva

Ageno

2019

Budd-Chiari Syndrome

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Flow cytometry screening for paroxysmal nocturnal hemoglobinuria: A single‐center experience in Saudi Arabia

Cited by 9 publications

References 23 publications

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

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

Paroxysmal nocturnal haemoglobinuria, diagnosis and haematological findings, first report from Iran, model for developing countries

Epidemiology of Budd–Chiari Syndrome

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