Progression in Ph-Chromosome-Negative Myeloproliferative Neoplasms: An Overview on Pathologic Issues and Molecular Determinants

Cancers

Clara Bertuzzi¹  Elena Sabattini¹  Claudio Agostinelli¹  Carlo Alberto Sagramoso Sacchetti¹  Francesca Palandri²  Umberto Gianelli³  Marco Pizzi⁴ 

[1] Haematopathology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy;Istituto di Ematologia “Seragnoli” IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy;Pathology Unit, Department of Pathophysiology and Transplantation, University of Milan and IRCCS Fondazione Ca’ Granda, Ospedale Maggiore Policlinico, 20122 Milan, Italy;Surgical Pathology and Cytopathology Unit, Department of Medicine—DIMED, University of Padua, 35121 Padua, Italy;

关键词: myeloproliferative neoplasms;    WHO classification;    progression;    fibrosis;    leukemia;   

DOI  :  10.3390/cancers13215531

来源: DOAJ

【 摘 要 】

Progression in Ph-chromosome-negative myeloproliferative neoplasms (MPN) develops with variable incidence and time sequence in essential thrombocythemia, polycythemia vera, and primary myelofibrosis. These diseases show different clinic-pathologic features and outcomes despite sharing deregulated JAK/STAT signaling due to mutations in either the Janus kinase 2 or myeloproliferative leukemia or CALReticulin genes, which are the primary drivers of the diseases, as well as defined diagnostic criteria and biomarkers in most cases. Progression is defined by the development or worsening of marrow fibrosis or the progressive increase in the marrow blast percentage. Progression is often related to additional genetic aberrations, although some can already be detected during the chronic phase. Detailed scoring systems for clinical usage that are mostly applied in patients with primary myelofibrosis have been defined, and the most recent ones include cytogenetic and molecular parameters with prognostic significance. Additional different clinic-pathologic changes have been reported that may occur during the course of the disease and that are, at present, classified as WHO-defined types of progression, although they likely represent such an event. The present review is meant to provide an updated overview on progression in Ph-chromosome-negative MPN, with a major focus on the pathologic side.

【 授权许可】

Unknown