Challenges of diagnosing and managing pre-fibrotic myelofibrosis: A case-based and practical approach.

Pre-Fibrotic Myelofibrosis is a frequently under-recognised entity that has distinct features separate to those of both Essential Thrombocythaemia and overt Primary Myelofibrosis. Misdiagnosis is relatively common due to subtle differences in bone marrow trephine morphology and multidisciplinary approaches are required. The clinical phenotype and disease course is heterogeneous and hence management approaches tend to vary widely.

Diagnostic and management strategies for Myeloproliferative Neoplasm-Unclassifiable (MPN-U): An international survey of contemporary practice.

Myeloproliferative Neoplasm-Unclassifiable (MPN-U) is defined as an MPN that fails to meet the diagnostic criteria for any of the other defined classical or 'non-classical' MPNs. The reported incidence is variable, dependent on appropriate recognition, and the clinical course can be highly variable ranging from an indolent disorder through to an aggressive disease course with a significant risk of thrombosis, bulky splenomegaly, and debilitating symptom burden. Clinicians frequently manage these conditions according to the clinical concerns e.

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

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.