Canterbury DHB

Context

Primary myelofibrosis (PMF) and MF secondary to ET/PRV

When considering the decision whether or not to recommend allogeneic transplantation the benefits, risks and alternative treatments must be considered as well as the natural history of PMF if untreated. Various risk scores have been devised including the dynamic IPSS (DIPSS) and the DIPSS-plus.

Transplant-eligible patients <45 years with an IPSS of int-2 or high, especially with transfusion dependence and/or adverse cytogenetics should be considered for conventional myeloablative SCT

Transplant-eligible patients >45 years with an IPSS of int-2 or high, especially with transfusion dependence and/or adverse cytogenetics, and an HSCT-CI ≥3 should be considered for reduced intensity SCT

Outcomes vary greatly between centres but for example, in the CIBMTR registry of 289 patients transplanted between 1989-2002, median age 47 years (18-73) the 100-day TRM was 18% for MSD, 35% for MUD and 19% for MMUD. 5-year OS was 37%, 30% and 40% respectively.

See Primary Myelofibrosis (PMF) in the MPD section.

Recommended reading:

About this Canterbury DHB document (10193):

Document Owner:

Andrew Butler (see Who's Who)

Issue Date:

December 2016

Next Review:

December 2018

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 10193