Canterbury DHB

Context

Prognosis

There are several prognostic scores to predict outcomes in PMF as below. DIPSS plus is a modern prognostic model which incorporates molecular information. See Rumi E, et al. (2017),1 table 6 page 9.

It is well established that the driver mutation has an impact on outcome in PMF as per the figure below:

Patients with mutated CALR and MPL genes have a favourable outcome, compared to those with JAK2 mutations, but patients who are triple negative appear to have the worst outcome, see Rumi E, et al. (2017),1 figure 4 page 10.

Vannucchi AM, et al. (2014)2 described a mutation-enhanced International Prognostic Scoring System (MIPSS) for Primary Myelofibrosis, including the mutations of:

In this scoring system, the following were found to be significant risk factors for poor survival:

By calculation of MIPSS, four risk groups are clearly delineated:

See Vannucchi AM, et al. (2014),2 figure 1 for:

Leukemia-free survival was also predicted by high (p<0.01) and intermediate 1/2 (p<0.01) risk MIPSS.

An extended molecular panel may be useful, especially when considering the benefit of allogeneic transplant. As of July 2018, extended molecular testing is not available in New Zealand, but the required genes for MIPSS can be requested from the Peter MacCallum Cancer Centre in Melbourne, Australia (myeloid panel cost approx. AUD600.00 + transport).

About this Canterbury DHB document (6243):

Document Owner:

Sean Macpherson (see Who's Who)

Issue Date:

August 2018

Next Review:

April 2021

Keywords:

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

Topic Code: 6243