Current opinion in hematology · 2025
Contemporary data in myeloproliferative neoplasm-unclassifiable: mutational landscape and management of the 'unclassifiable'.
СтатьяОбзорПоддержка исследования: вне правительства СШАПоддержка NIH: внешняя программаНазвание и аннотация: Перевод в очереди
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Oganesyan Artem, Madero-Marroquin Rafael, Patel Anand A
Аннотация
PURPOSE OF REVIEW: Myeloproliferative neoplasm-unclassifiable (MPN-U) is a heterogenous subtype of Philadelphia-chromosome negative (Ph-) MPN that comprises 5-20% of Ph- MPN cases. Given the clinical and molecular variability of presentation, there are no clear data regarding risk stratification and management of MPN-U. In this review, we aim to summarize the current evidence regarding MPN-U with regards to diagnosis, risk stratification, and treatment approaches.
RECENT FINDINGS: Current diagnostic criteria require exclusion of other chronic myeloid malignancies along with identifying the typical pathologic findings of an MPN in order to diagnose MPN-U. While there are not dedicated prognostic scores regarding thrombotic risk and survival specifically for MPN-U, phenotype-agnostic scores may have utility. In addition, the Dynamic International Prognostic Scoring System (DIPSS)+ score has demonstrated that it can identify higher-risk MPN-U. There are no consensus treatment recommendations available for MPN-U.
SUMMARY: When considering management of MPN-U, for patients in the cellular phase, we consider cytoreduction in patients identified as high-risk, for fibrotic-phase disease, we consider JAK inhibition for splenomegaly/constitutional symptoms and allogeneic transplantation for higher-risk patients, and for accelerated/blast-phase disease we employ hypomethylating-agent based approaches with the intent of allogeneic transplant for eligible patients.
RECENT FINDINGS: Current diagnostic criteria require exclusion of other chronic myeloid malignancies along with identifying the typical pathologic findings of an MPN in order to diagnose MPN-U. While there are not dedicated prognostic scores regarding thrombotic risk and survival specifically for MPN-U, phenotype-agnostic scores may have utility. In addition, the Dynamic International Prognostic Scoring System (DIPSS)+ score has demonstrated that it can identify higher-risk MPN-U. There are no consensus treatment recommendations available for MPN-U.
SUMMARY: When considering management of MPN-U, for patients in the cellular phase, we consider cytoreduction in patients identified as high-risk, for fibrotic-phase disease, we consider JAK inhibition for splenomegaly/constitutional symptoms and allogeneic transplantation for higher-risk patients, and for accelerated/blast-phase disease we employ hypomethylating-agent based approaches with the intent of allogeneic transplant for eligible patients.