Screening for paroxysmal nocturnal hemoglobinuria (PNH) in patients presenting with cerebral sinovenous thrombosis (CSVT): Results of a FLAER based flowcytometry study in Indian patients

Sharma, Saniya; Ahluwalia, Jasmina; Atreja, Charu Batra; Modi, Manish; Sachdeva, Manupdesh Singh; Kumar, Narender; Bose, Parveen; Bose, Smarajit; Uppal, Varun; Lal, Vivek; Varma, Neelam

doi:10.1007/s11239-019-01982-y

Cited by 3 publications

(2 citation statements)

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“…A study from Chandigarh, India, conducted in 2014, showed two out of 142 patients with intra-abdominal thrombosis as having large PNH clones 14 . Another study from Chandigarh, India, published in 2020 did not detect PNH phenotype in any of the 180 patients with cerebral sinovenous thrombosis 15 . A study from Turkey published in 2020 conducted on patients with idiopathic portal vein thrombosis showed PNH clone size between 3.02 and 4.62 per cent in four out of 112 patients 16 .…”

Section: Resultsmentioning

confidence: 89%

Detection of paroxysmal nocturnal haemoglobinuria clones in cases of deep vein thrombosis in a tertiary care centre, western Rajasthan

Passey

Purohit

2022

Indian J Med Res

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Background & objectives: Paroxysmal nocturnal haemoglobinuria is a rare acquired disease characterized by bone marrow failure, intravascular haemolysis and thrombophilia. Thrombosis is the deadliest complication of paroxysmal nocturnal haemoglobinuria (PNH). The present study was conducted to study the prevalence of PNH in cases of deep vein thrombosis (DVT) which was previously undocumented from western Rajasthan. Methods: In the present cross-sectional study, 61 adult patients with DVT were tested using flow cytometry to detect PNH clones. Blood samples were processed using fluorescein-labelled proaerolysin, CD14, CD24, CD33 and CD45 panels for granulocytes and monocytes and CD59 and CD235a panel for red blood cells. Results: Three cases (4.92%) having large clones on monocytes as well as granulocytes, which fulfilled the diagnostic criteria of PNH were detected. Further, three cases (4.92%) showed small clones on both granulocytes and monocytes. Nine (15%) cases showed small clones only on granulocytes, and 11 (18%) cases showed small clones only on monocytes. Interpretation & conclusions: The results of the present study suggest that a higher proportion of patients had PNH in western Rajasthan compared to previously reported studies from elsewhere. It is suggested that PNH testing should be added to the procoagulant work-up panel in institutions of this region where it is not routinely done. This provides an otherwise missed opportunity to diagnose this disorder. Eculizumab may be employed, which is effective in reducing thrombophilic events in cases of PNH.

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Section: Resultsmentioning

confidence: 89%

Detection of paroxysmal nocturnal haemoglobinuria clones in cases of deep vein thrombosis in a tertiary care centre, western Rajasthan

Passey

Purohit

2022

Indian J Med Res

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“…Small to moderate PNH clones are frequently found in patients with acquired aplastic anemia (AA) (8,9) and occasionally with myelodysplastic syndromes (MDSs) (10). PNH clones can also be found in patients with unexplained asymptomatic cytopenia (11,12), thrombosis at unusual sites (13)(14)(15), pain associated with smooth muscle dystonia (16), and a variety of rare Coombs-negative hemolysis scenarios (17-20); however, it is important to mention that the traditional inclusion of PNH only in Coombs-negative hemolytic conditions should be reviewed (21). Furthermore, in bone marrow failure, the finding of a PNH clone can provide an important diagnostic clue about the immunemediated pathogenesis of underlying marrow aplasia (22,23).…”

Section: Introductionmentioning

confidence: 99%

Swiss Survey on current practices and opinions on clinical constellations triggering the search for PNH clones

et al. 2023

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This national survey investigated the current practice in Switzerland by collecting participants' opinions on paroxysmal nocturnal hemoglobinuria (PNH) clone assessment and clinical practice.AimThis study aimed to investigate clinical indications prompting PNH clones' assessment and physician's accessibility of a flow cytometry facility, and also to understand clinical attitudes on the follow-up (FU) of patients with PNH clones.MethodsThe survey includes 16 multiple-choice questions related to PNH and targets physicians with a definite level of experience in the topic using two screener questions. Opinion on clinical management was collected using hypothetical clinical situations. Each participant had the option of being contacted to further discuss the survey results. This was an online survey, and 264 physicians were contacted through email once a week for 5 weeks from September 2020.ResultsIn total, 64 physicians (24.2%) from 23 institutions participated (81.3% hematologists and 67.2% from university hospitals). All had access to flow cytometry for PNH clone testing, with 76.6% having access within their own institution. The main reasons to assess for PNH clones were unexplained thrombosis and/or hemolysis, and/or aplastic anemia (AA). Patients in FU for PNH clones were more likely to be aplastic anemia (AA) and symptomatic PNH. In total, 61% of the participants investigated PNH clones repetitively during FU in AA/myelodysplastic syndromes patients, even when there was no PNH clone found at diagnosis, and 75% of the participants tested at least once a year during FU. Opinions related to clinical management were scattered.ConclusionThe need to adhere to guidelines for the assessment, interpretation, and reporting of PNH clones emerges as the most important finding, as well as consensus for the management of less well-defined clinical situations. Even though there are several international guidelines, clear information addressing specific topics such as the type of anticoagulant to use and its duration, as well as the indication for treatment with complement inhibitors in some borderline situations are needed. The analysis and the discussion of this survey provide the basis for understanding the unmet needs of PNH clone assessment and clinical practice in Switzerland.

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Prothrombin G20210A Mutation is Rare but not Absent Among North Indian Patients with Thromboembolic Events

Satyarthi,

Ray,

Kumar

et al. 2024

Indian J Hematol Blood Transfus

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Screening for paroxysmal nocturnal hemoglobinuria (PNH) in patients presenting with cerebral sinovenous thrombosis (CSVT): Results of a FLAER based flowcytometry study in Indian patients

Cited by 3 publications

References 32 publications

Detection of paroxysmal nocturnal haemoglobinuria clones in cases of deep vein thrombosis in a tertiary care centre, western Rajasthan

Detection of paroxysmal nocturnal haemoglobinuria clones in cases of deep vein thrombosis in a tertiary care centre, western Rajasthan

Swiss Survey on current practices and opinions on clinical constellations triggering the search for PNH clones

Prothrombin G20210A Mutation is Rare but not Absent Among North Indian Patients with Thromboembolic Events

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