BCR-ABL1 Negative Myeloproliferative Neoplasms

Dear Colleagues,

BCR-ABL1-negative myeloproliferative neoplasms (MPNs) are a group of hematopoietic stem-cell-derived clonal myeloid diseases, featured by an inflammatory condition affecting patient quality of life and supporting tumor progression.

The presence of a driver mutation in the JAK2, CALR or MPL gene in the majority of patients plays a key role not only in diagnosis, but also in evaluation of prognosis that can be further modified by the eventual presence of other subclonal mutations.

Both driver and subclonal mutations are now taken into consideration in new prognostic scoring systems and may be better investigated using novel molecular biology techniques, such as next-generation sequence (NGS).

Finally, the discovery in MPN patients of mutations affecting JAK signaling as a major pathogenic mechanism of MPNs has prompted the development of JAK inhibitors (JAKi), which are now in the standard armamentarium to fight these diseases. Recently, other drugs targeting different cellular targets have been investigated with promising results, and combinatorial therapies with JAKi have begun to be envisioned. However, allogeneic bone marrow transplantation is still a mainstay in the therapy of young patients.

This Special Issue, “BCR-ABL1-Negative Myeloproliferative Neoplasms”, will cover a selection of recent research topics and current review articles in the field of diagnosis, prognosis, and therapy of these diseases.

Prof. Giuseppe A. Palumbo
Guest Editor

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