Winter 2017 Vol 2. No. 1

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We publish this newsletter quarterly, with information on what’s new in MPN care and research. Each issue we bring together MPN advances and news, and keep you up to date with the work of the Canadian MPN Group. Comments, ideas and suggestions can be sent to info@mpncanada.com

What kind of MPN patients do you see?

We see 150-175 new MPN patients a year. We treat patients with all MPNs but the majority of our referrals for are patients with myelofibrosis (MF) due to the greater complex-ity of the disease and these patients are more likely to be referred for a second opinion at a specialist MPN centre like ours.

MPN team

We are fortunate to have a diverse clinical and research team. We have three staff physicians supporting the program (Dr. Vikas Gupta, Dr. Dawn Maze and Dr. Hassan Sibai) a clinical re-search fellow (Dr. Caroline McNamara), translational research

Spotlight – The Elizabeth and Tony Comper MPN Program at Princess Margaret Cancer Centre, Toronto

fellow (Dr. Jenny Ho) and a clinical nurse specialist (Nancy Siddiq, RN) supporting the clinical work of the program. Our research support staff include a program manager (Rebecca Devlin), database manager (Jayalakshmi Ramanna) and clini-cal coordinator (Andrea Arruda)

What is the program doing to improve MPN care?

Many of our patients travel a significant distance for their MPN care at Princess Margaret and this can be burdensome. To address this we have established excellent links with hematologists throughout Ontario so that our MPN patients can receive the more routine aspects of their MPN care (such as blood transfusion or phlebotomy) closer to their home but

Newsletter

Every newsletter we highlight a leading centre for MPN care and research in Canada. In this issue we look at the Elizabeth and Tony Comper MPN Program at Princess Margaret Cancer Centre in Toronto.

Some of the MPN Program teamLeft to right: Dr. Jenny Ho, Dr. Caroline McNamara, Andrea Arruda, Dr. Vikas Gupta, Rebecca Devlin, Dr. Waleed Aldauij, Dr. Hassan Sibai, Nancy Siddiq (RN)

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Spotlight... continued from page 1

maintain access to MPN expert care at Princess Margaret. Our clinical nurse specialist, Nancy Siddiq RN, helps coordinates this and serves as a valuable direct contact point for MPN patients at Princess Margaret.

Do patients receive genetic testing?

All patients seen at the MPN clinic are invited to participate in a voluntary research project that tests for mutations in a panel of genes. As this is a research project, the results are not expected to affect a patient’s care but this research helps us learn more about MPNs and improve care in the future.

What clinical trials are active at Princess Margaret?

The MPN clinic at Princess Margaret currently has several trials for patients with MF. These include a phase 2 trial for the novel drug PRM-151, which is being analyzed to see whether in can affect bone marrow fibrosis (NCT01981850; no longer enrolling new patients). A Phase 1/2 trial for another novel drug, SL-401, is also open. This trial is for those with high-risk MPNs (MF, mastocytosis, advanced symptomatic hypereo-sinophilic disorder and chronic myelomonocytic leukemia) (NCT02268253). A phase 3 trial examining early treatment with ruxolitinib in patients with MF and high-risk mutations is also currently recruiting (NCT02598297). Full details of clinical trials across Canada can be found at www.mpncanada.com

What MPN research is going on in your program?

In our multidisciplinary team we have a number of exciting projects going on in the areas of patient education, shared care, clinical studies and basic research. In collaboration with colleagues at Toronto General Hospital, we initiated a study to screen MPN patients for portal vein thrombosis and portal hypertension to investigate how prevalent these serious complications are in MPN patients. In 2016 we launched an MPN patient registry (NCT02760238). All MPN patients seen in our clinic are invited to participate in this voluntary study, where MPN clinical and genetic data are collected at multiple time points. This registry will be a valuable resource for future research to learn more about MPNs.

What does being part of the Canadian MPN Group mean to you?

Shared care between specialist centres and local hematolo-gists is crucial in a rare disease like MPN. Working with colleagues in the Group to help in MPN education of health care professionals is vital to facilitate this model.

ReferralsContact: Nancy Siddiq, RNTel: 416-946-4501 x3037Fax: 416-946-2900

Vikas Gupta MD, FRCP, FRCPath

Dr. Gupta is site lead for the Elizabeth and Tony Comper MPN Program, and a member of the Leukemia/MPN Program at Princess Margaret Cancer Centre. He is the founding member and president of the Canadian MPN Group. His clinical and research activities are focused on developing novel transplant and non-transplant based treatment strategies for patients with myeloid malignancies, in particular myeloproliferative neoplasms.

Dawn Maze MD, FRCPC, MSc

Having completed her Medical Degree and Internal Medicine training at Memorial University, Dr. Maze did a Hematology Fellowship at Queen’s University during which time she completed a Master of Science in Clinical Epidemiology. She joined the Leukemia Program at the Princess Margaret Cancer Centre in 2016. Her research activities are focused on optimizing transfusion practices

Referrals accepted from physicians only and must be faxed to New Patient Referrals Department, marked “for the attention of Nancy Siddiq”.

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Hassan Sibai MD, FRCPC

After completing a fellowship at the Ameri-can University of Beirut in Lebanon, he joined the Leukemia Program at the Princess Marga-ret Cancer Centre as a fellow, where he is now a Staff Physician in the Leukemia Program. Dr. Sibai actively sees patients referred to the MPN Program and has a focus in innovations in malignant hematology education.

Auro Viswabandya MD, DM

Dr. Viswabandya currently is a staff physician in Allogeneic Stem Cell Transplantation divi-sion at the Princess Margaret Cancer Centre and Assistant Professor, Department of Medicine at the University of Toronto. He is actively involved in allogeneic stem cell transplantation in patients with myeloproliferative syndromes. His clinical and research activities are focused on haploidentical stem cell transplantation in patients with hematological malignancies.

Meeting updates

Two randomized trials of hydroxyurea in PV and ET

1. Interim analysis of MPN-RC112: peg IFN-alpha 2 vs hydroxyurea in PV and ET (NCT01258856) – Mascarenhas et al

2. Results of PROUD-PV: ropeginterferon alfa-2b vs hydroxyurea in PV – Gisslinger et al

Neither of these trials showed a difference in response at 12 months. However, longer term follow up of both study populations is required to assess differences in response and toxicity between these two agents over time.

Results of the PERSIST-2 phase 3 study of pacritinib versus best available therapy in MF– Mascarenhas et al

Development of pacritinib was put on clinical hold by the Federal Drug Authority (FDA) but partial results were presented at this meeting. Patients treated with pacritinib had significant reduction in spleen size compared to those treated with best available treatment (including ruxolitinib). However, serious treatment-emergent adverse events occurred, including cardiac arrest, congestive heart failure

and atrial fibrillation. The FDA has lifted the clinical hold on pacritinib and we should see new studies in the future.

MPD-RC 114 trial stage 1 results – ruxolitinib in transplant for MF – Gupta et alThis study (NCT01790295) investigated the potential of using ruxolitinib prior to undergoing bone marrow trans-plant for MF. Stage 1 results suggested use of ruxolitinib prior to transplant was safe and there was no adverse impact of ruxolitinib on transplant outcomes.

Combination therapies with ruxolitinib

1. Ruxolitinib in combination with 5-azayctidine as therapy for patients with MF (NCT01787487) – Daver et al

2. Safety and efficacy of combined ruxolitinib and decitabine in patients with blast phase MPN and post-MPN AML (phase 1, MPN-RC 109) – Rampal et al

Ruxolitinib appears to be safe with both of these combina-tions. Future randomized trials are needed to compare each of these combinations to ruxolitinib monotherapy.

Highlight from the American Society of Hematology (ASH) Annual Meeting 2016

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013x_VisualServices@uhn.on.ca

EDITORIAL COMMITTEE: Lynda Foltz, MD, FRCPC

Vikas Gupta, MD, FRCP, FRCPath

Christopher Hillis, MD, MSc, FRCPC

Shireen Sirhan, MD, FRCPC

EDITOR:

Rebecca Devlin, PhD

The Canadian MPN Group Newsletter is published quarterly

For further information, please contact info@mpncanada.com

ACKNOWLEDGEMENTSWe would like to acknowledge the support of Novartis Oncology for making this newsletter possible and staff at University Health Network, Visual Servies for their help in the preparation of this newsletter.

CANADIAN MPN GROUP INFO:Tel.: 613-748-9613

Fax: 613-748-6392

Email: info@mpncanada.com

Registered Charity: 83976 6136 RR0001

Website: mpncanada.com

Twitter: @MPNGroupCanada

Facebook: facebook.com/ CanadianMPN

The Elizabeth and Tony Comper MPN Program at Princess Margaret Cancer Centre, Toronto, developed a booklet to help MPN patients and their families attending their clinic learn about their diagnosis.

The booklet covers the basics on MPNs, including:

• What MPNs are

• What symptoms to expect

• Living with an MPN

• Treatments you may be offered

• Support groups and where to get more information

The booklet is available on the Canadian MPN Group website http://www.mpncanada.com/living-with-mpn/other-useful-resources/

We welcome your feedback on the booklet! Contact us at info@mpncanada.com The booklet is only intended for general information purposes and not to diagnose, treat, cure or prevent disease. Please consult your physician for medical advice.

The MPN e-SIMPLE app was developed with the support of Novartis. The app provides data, resources and guidance to assist Canadian physicians in daily management of MPNs. The goal is to improve the management of MPNs across Canada by providing point-of-care guidance to our colleagues on therapeutic approaches for each MPN patients.

The app includes disease risk calculators (IPSS, DIPSS, DIPSS Plus and IPVS), resources for MPN diagnosis and risk assessment, and information on treatment options. At present the app covers MF and PV, with ET coming soon. MPN e-SIMPLE can be used through your web browser or downloaded (Android & iOS, optimized for tablets) at www.mpnesimple.ca

MPNs: A Guide for Patients & Families

MPN e-SIMPLE App