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.
Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre / Deschamps, P.; Moonim, M.; Radia, D.; Curto-Garcia, N.; Woodley, C.; Bassiony, S.; O'Sullivan, J.; Harrington, P.; Raj, K.; Francis, Y.; Kordasti, S.; Ali, S.; Harrison, C. N.; Mclornan, D. P.. - In: BRITISH JOURNAL OF HAEMATOLOGY. - ISSN 0007-1048. - ELETTRONICO. - 193:4(2021), pp. 792-797. [10.1111/bjh.17375]
Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre
Kordasti S.Writing – Review & Editing
;
2021-01-01
Abstract
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
