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.
DOIAbstract
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 leucocy...
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