Management of myelofibrosis after ruxolitinib failure.

Abstract:

:Over the last decade, the Janus kinase1/2 (JAK1/2) inhibitor ruxolitinib has emerged as a cornerstone of myelofibrosis (MF) management. Ruxolitinib improves splenomegaly and symptoms regardless of driver mutation status, and confers a survival advantage in patients with intermediate-2/high risk MF. However, cytopenias remain problematic, and evidence for a robust anti-clonal effect is lacking. Furthermore, the median duration of spleen response to ruxolitinib in clinical trials is approximately 3 years, and ruxolitinib does not appear to affect the risk of leukemic transformation. There is no therapy approved specifically for patients whose disease 'progresses' on ruxolitinib, defining which remains challenging. The recent regulatory approval of the JAK2 inihibitor fedratinib partially fulfills this unmet need, but much remains to be done. Other JAK inhibitors and a plethora of novel agents are being studied in the ruxolitinib 'failure' setting, as well as 'add-on' therapies to ruxolitinib in patients having a 'sub-optimal' response.

journal_name

Leuk Lymphoma

journal_title

Leukemia & lymphoma

authors

Bose P,Verstovsek S

doi

10.1080/10428194.2020.1749606

subject

Has Abstract

pub_date

2020-08-01 00:00:00

pages

1797-1809

issue

8

eissn

1042-8194

issn

1029-2403

journal_volume

61

pub_type

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