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Longitudinal trends of peripheral blood counts in polycythaemia vera and essential thrombocythemia patients in the UK.

ABSTRACT: There is sparse evidence of how well haematological targets are met in practice for essential thrombocythemia (ET) and polycythaemia vera (PV) patients. Patient data was collected between 2008 and 2020 from two UK NHS Trusts for ET and PV patients. Longitudinal changes in peripheral blood counts, including the proportion of patients meeting peripheral blood count remission, was modelled. Relative risk of cardiovascular-related events for patients achieving remission within 3-months was estimated. A total of 620 ET and 429 PV patients were analysed. For high-risk patients, haematological parameters decreased in the first months of observation then stabilised within normal reference ranges until year 5. Total time spent in peripheral blood count remission was 39.2% for ET and 29.1% for PV. A lower proportion of ET patients reached target platelet counts (48.3%) compared to WBC (79.1%), whilst PV patients were less likely to reach target haematocrit levels (56.9%) compared to platelets (77.3%) or WBC (74.6%). There was no statistically significant association between reaching target blood counts within 3-months and cardiovascular risk. Complete haematological remission remains a challenging target in managing PV and ET, however this study was unable to show statistically-significant evidence that this was associated with increased risk of cardiovascular events.

SUBMITTER: Carpenter L

PROVIDER: S-EPMC9421951 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature

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Longitudinal trends of peripheral blood counts in polycythaemia vera and essential thrombocythemia patients in the UK.

Carpenter Lewis L Rockenschaub Patrick P Hatton Grace B GB D'Abrantes Sofia S Sims Edward E Scott-Ram Nicholas N Ducès Aurélie A Emanuel Gabrielle G Mead Adam J AJ Drummond Mark W MW Lipunova Nadezda N

EJHaem 20220629 3

There is sparse evidence of how well haematological targets are met in practice for essential thrombocythemia (ET) and polycythaemia vera (PV) patients. Patient data was collected between 2008 and 2020 from two UK NHS Trusts for ET and PV patients. Longitudinal changes in peripheral blood counts, including the proportion of patients meeting peripheral blood count remission, was modelled. Relative risk of cardiovascular-related events for patients achieving remission within 3-months was estimated ...[more]

PMID: 36051073

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Project description:Polycythemia vera (PV) and essential thrombocythemia (ET) are rare myeloproliferative neoplasms (MPN) in children, adolescents and young adults. No recommendations are available concerning these patients' management. Transposing to children the knowledge established in adult patients is not acceptable. For a better understanding of difficulties encountered by pediatricians and adult hematologists, we conducted a national practice analysis concerning follow-up of patients under 18 diagnosed with ET or PV, in France. Then, we present data from a multicentric, descriptive, retrospective study, including 17 patients with ET or PV, diagnosed under 18, coming from 7 hematopediatric departments in France. Interviewed physicians reported a lack of expertise and theoretical training in the hematological field to diagnose and follow children with MPNs. Data from 17 patients (15 ET, 2 PV) confirmed a high proportion of asymptomatic patients at the time of diagnosis (41%). Proportion of "triple-negative" patients (59%) was higher than in adult cohorts. 60% of patients underwent a bone marrow biopsy and 31% of cases were discussed during a multi-disciplinary staff meeting. 76.5% patients were treated, with a high frequency of antithrombotic and cytoreductive drugs. No complications were observed during the 45 months of median follow-up.ConclusionPhysicians insisted on the need for training. Only the accumulation of descriptions of MPNs in children will lead to a better management of these diseases. Considering the small proportion of pediatric patients with complications after diagnosis, rapid therapeutic de-escalation seems essential to consider during the follow-up in a close collaboration with adult hematologists.What is known• Myeloproliferativ neoplams are rare and chronic deseases, most of the time affecting adults but also found in few pediatric patients. • There are no recommendations for the diagnosis, therapeutic management or follow-up of children with polycythemia vera or essential thrombocythemia.What is new• Find out how adult haematologists and paediatricians feel about the management and follow-up of paediatric patients with myeloproliferative syndrome through a national practice analysis. • Description of the "real life" follow-up of children with polycythemia vera or essential thrombocythemia in France.

Dataset Information

Longitudinal trends of peripheral blood counts in polycythaemia vera and essential thrombocythemia patients in the UK.

Publications

Longitudinal trends of peripheral blood counts in polycythaemia vera and essential thrombocythemia patients in the UK.

Similar Datasets

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