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Patients with myeloproliferative neoplasms (MPN) are known to have higher incidence of nonhematological second primary malignancies (SPM) compared to general population. In the MYSEC study on 781 secondary myelofibrosis (SMF) patients, the incidence of SPM after SMF diagnosis resulted 0.98/100 patient‐years. When including non‐melanoma skin cancers (NMSC), the incidence arose to 1.56/100 patient‐years. In SMF, JAK inhibitor treatment was associated only with NMSC occurrence. Then, we merged the MYSEC cohort with a large dataset of PV and ET not evolving into SMF. In this subanalysis, we did not find any correlation between SPM and SMF occurrence. These findings highlight the need of studies aimed at identifying MPN patients at higher risk of SPM. [ABSTRACT FROM AUTHOR]

The article presents a study which examined the prognostic relevance of monocytosis as effective and independent predictor of inferior survival in primary myelofibrosis (PMF). Also cited are the use of Dynamic International Prognostic Scoring System (DIPSS)-plus as most effective PMF prognostic tool, and the use of univariate analysis to analyze the relationship of confidence interval (CI), absolute monocyte count (AMC), and hazard ratio (HR) to inferior overall survival in patients.

The article focuses on a study related to prefibrotic versus overtly fibrotic primary myelofibrosis. Topics discussed include presence of higher hemoglobin, higher platelet count, higher incidence of thrombocytosis in pre-primary myelofibrosis; lower incidence of splenomegaly; and WHO-defined diagnosis of early/prefibrotic primary myelofibrosis.

The article presents a study which aims at analyzing prognostic effect of cytogenetic information in polycythaemia vera (PV), in terms of myelofibrosis‐free survival (MFFS) and thrombosis‐free survival (TFS), in context of other risk factors, including adverse somatic mutations. It mentions that PV is a myeloproliferative neoplasm (MPN) that is characterized by increased red cell mass and JAK2 mutations. It presents information on mutation screening for adverse mutation.

Purpose of Review: The purpose of this review is to help doctors in the management of patients with post-polycythemia (PPV) and post-essential thrombocythemia (PET) myelofibrosis (MF) facing diagnostic criteria, prognostication, and treatment possibilities.Recent Findings: Diagnostic criteria of primary myelofibrosis (PMF) have been recently updated from the WHO classification. A clear-cut distinction between pre-fibrotic and overt PMF has been done. Concerning PPV and PET MF, the criteria come from 2008. Prognostication of PMF has been well established on clinical criteria, but recent molecular acquisitions will improve the strategy. For PPV and PET MF, the new MYSEC-PM is helpful for prediction of survival. JAK2-inhibitors and stem cell transplant are the two critical therapeutic approaches in myelofibrosis.Summary: Differences between PMF and SMF substantiate the efforts underway to adequately stratify SMF patients with ad hoc prognostic tools and to use such categorization to evaluate available treatment modalities. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discusses the study on the clinical spectrum and outcomes of autoimmune diseases (AID) and/or systemic inflammatory syndromes (SIS) in patients with chronic myelomonocytic leukemia (CMML).

Myeloproliferative neoplasms associated with FIP1L1- PDGFR rearrangements represent a rare subset of myeloid and lymphoid malignancies, characterised by the presence of eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1 genes. The fusion product of such genes is a tyrosine kinase oncoprotein sensitive to imatinib, which to date results to be the standard of care for FIP1L1- PDGFRA-positive chronic myeloproliferative disorders with eosinophilia. However, the coexistence of FIP1L1- PDGFRA rearrangement associated with acute myeloid leukaemia is extremely rare. Here, we report a rare case of FIP1L1- PDGFRA-positive acute myeloid leukaemia, with marked peripheral blood and bone marrow eosinophilia, treated with low dose of imatinib monotherapy, achieving a rapid and long-lasting complete cytologic and molecular remission, without need for intensive chemotherapy. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented on the effectiveness of the nilotinib drugs for treatment of eosinophilic myeloproliferative neoplasm disease.

A letter to the editor is presented on diagnosis of polycythemia vera.