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 11·25years (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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