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Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre.
Deschamps, Paul; Moonim, Mufaddal; Radia, Deepti; Curto-Garcia, Natalia; Woodley, Claire; Bassiony, Sarah; O'Sullivan, Jennifer; Harrington, Patrick; Raj, Kavita; Francis, Yvonne; Kordasti, Shahram; Ali, Sahra; Harrison, Claire N; McLornan, Donal P.
Afiliação
  • Deschamps P; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Moonim M; Service d'Hématologie, Hôpital Cochin Assistance Publique Hôpitaux de Paris, Paris, France.
  • Radia D; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Curto-Garcia N; Histopathology Department, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Woodley C; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Bassiony S; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • O'Sullivan J; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Harrington P; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Raj K; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Francis Y; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Kordasti S; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Ali S; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • Harrison CN; Department of Haematology, Guy' s and St Thomas' NHS Foundation Trust, London, UK.
  • McLornan DP; Systems Cancer Immunology, Comprehensive Cancer Centre, King's College London, London, UK.
Br J Haematol ; 193(4): 792-797, 2021 05.
Article em En | MEDLINE | ID: mdl-33751548
Myeloproliferative neoplasm-unclassifiable (MPN-U) presents an MPN-type phenotype that fails to meet diagnostic criteria for other MPN variants. Variability in the clinicopathological phenotypes presents many challenges. Amongst a registry cohort of 1512 patients with MPN, 82 with MPN-U were included, with a median (range) age of 49·7 (13-79) years. Albeit heterogeneous, common presentation features included raised lactate dehydrogenase, thrombocytosis and clustered/pleomorphic megakaryocytes on trephine biopsy. Thrombosis was common (21%), necessitating vigilance. The median event-free survival was 11·25 years (95% confidence interval 9·3-not reached), significantly shortened in cases with lower platelet counts (<500 × 109 /l) and a leucocytosis (≥12 × 109 /l) at presentation. Generation of potential MPN-U prognostic scores is required.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Hematológicas / Centros de Atenção Terciária / Transtornos Mieloproliferativos Tipo de estudo: Observational_studies / Prognostic_studies Limite: Adolescent / Adult / Aged / Female / Humans / Male / Middle aged País/Região como assunto: Europa Idioma: En Revista: Br J Haematol Ano de publicação: 2021 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Hematológicas / Centros de Atenção Terciária / Transtornos Mieloproliferativos Tipo de estudo: Observational_studies / Prognostic_studies Limite: Adolescent / Adult / Aged / Female / Humans / Male / Middle aged País/Região como assunto: Europa Idioma: En Revista: Br J Haematol Ano de publicação: 2021 Tipo de documento: Article