How I manage myeloproliferative neoplasm‐unclassifiable: Practical approaches for 2022 and beyond

作者：Donal P. McLornan, Rupen Hargreaves, Juan Carlos Hernández‐Boluda, Claire Harrison · 发表于：British Journal of Haematology · 年份：2022 · DOI：10.1111/bjh.18087 · 被引用次数：9 · 研究领域：Myeloproliferative Neoplasms: Diagnosis and Treatment、Eosinophilic Disorders and Syndromes、Chronic Myeloid Leukemia Treatments

Myeloproliferative neoplasm (MPN)-unclassifiable (MPN-U) or not otherwise specified represents a rare, poorly defined and heterogeneous group of MPNs. Disease incidence is difficult to define but likely represents close to 5% of all MPNs when strict World Health Organisation (WHO) criteria are applied. Dynamic review over time is required to assess if the disease can be re-classified into another MPN entity. A diagnosis of MPN-U leads to many challenges for both the patient and physician alike including lack of agreed monitoring and therapeutic guidelines, validated prognostic markers and licenced therapies coupled with exclusion from clinical trials. MPN-U has an inherent risk of an aggressive clinical course and transformation in some but who, and when to treat in the chronic phase, including identifying who may require more aggressive therapy at an earlier stage, remains elusive. Moreover, despite the significant thrombotic risk, there is no agreement on systematic primary thromboprophylaxis. We hereby provide a contemporary overview of MPN-U in addition to four illustrative cases providing our collective suggested approaches to clinical challenges.