• MPNRF | April 7, 2018

    Each year the American Society of Hematology (ASH) hosts their annual convention, where researchers, doctors, and organizations such as the MPN Research Foundation gather to discuss the ever-changing world of blood disorders and learn about the progress of research. The content below is a selection of MPN related takeaways from the convention this year, however, a more comprehensive review, written by MPNRF Scientific Advisory John Crispino will be available in early 2019. Be the first to see the review by clicking hereand signing up for our free updates!

    MPN and Pregnancy

    Pregnancy Outcomes in Patients with Myeloproliferative Neoplasms: A Systematic Review and Meta-Analysis In their meta-analysis of data on pregnant women with MPN and their outcomes, they found that patients who received low-dose aspirin during pregnancy had a 5-fold higher odds of a successful pregnancy than those managed with usual care and observation alone. Cytoreduction with INF improved the odds of live birth by nearly 4-fold. Maternal events were uncommon overall and no intervention was found to be beneficial. They caution that due to the population size they studied more work to confirm this is needed, and that every woman should consult closely with her OBGYN & a hematologist who specializes in MPN when making decisions about care.

    MPN in Younger Populations

    Once thought to be only a disease of the aging, there is wider recognition now that people of any age can be diagnosed with PV, ET or even MF. Here are some publications from this meeting on the subject:

    Myeloproliferative Neoplasms in Patients below 25 Years Old at Diagnosis: A Retrospective International Cooperative Work

    Myeloproliferative Neoplasms in Young Patients: The Mayo Clinic Experience with 361 Cases Age 40 Years or Younger

    Basic Science of MPN

    The oral sessions Basic Science: Mechanisms of Development and Progression allowed a variety of researchers to present their work. Of particular interest is Linda Resar’s study of the suspected epigenetic switch HMGA ½. Dr. Resar believes that silencing HMGA in AML cell lines from JAK+ patients disrupts progression. Her lab has developed a mouse model to test this theory further. Samuel Stoner described the hippo kinase activation process. Dr. Stoner believes that JAK2 and HIPPO cooperate to increase a pro-inflammatory cytokine environment. They are working on creating a mouse model to further test this.

    All oral session abstracts presented at this section are found here

    Myeloproliferative Neoplasm (MPN) Blastic Transformation Occurs at the Level of Hematopoietic Stem Cells

    MPN Therapies

    Our own scientific Advisor John Crispino, a Robert I. Lurie, MD, and Lora S. Lurie Professor of Medicine at Northwestern gave a talk at the European Hematology Association / American Society of Hematology joint symposium on GATA1 mutation and how it led him to test an Aurora Kinase Inhibitor – Alisertib – in myelofibrosis, to what appears so far to be good results. There was also a poster presented by Naseema Gangat on this drug titled “Alisertib (MLN8237), an Oral Selective Inhibitor of Aurora Kinase a, Has Clinical Activity and Restores GATA1 Expression in Patients with Myelofibrosis:

    An emerging drug – AVID200 – was the topic of a presentation by Ronald Hoffman. AVID200 is a TGFBeta Inhibitor which is believed to target fibrosis in particular and promote the growth and survival of health bone marrow cells. This trial is being opened as an investigator-initiated trial led by the MPN Research Consortium. We will communicate once it is open in the event it is something you want to discuss with your doctor.  

    PRM – 151 was also discussed by Srdan Verstovsek. Quoting the abstract “This long-term follow-up study of 18 pts with MF who received PRM-151 for a median of 31 months showed the drug to be well tolerated. An overall improvement in BM reticulin and collagen fibrosis grade, as well as reductions in MPN-SAF TSS and splenomegaly, were observed. In a subset of pts for whom bone marrow fibrocyte immunostaining data were available, a mean reduction in fibrocyte counts was observed, as well as improved bone marrow reticulin and collagen fibrosis grade. These data warrant confirmation in a larger controlled study.” This was part of a session on emerging therapies. All abstracts presented can be found here.

    The outcome of Patients with Myelofibrosis after Ruxolitinib Failure: Role of Disease Status and Treatment Strategies in 214 Patients


    Results from the Myeloproliferative Neoplasm Patient Care Survey: Patient Care Opportunities and Challenges The survey results suggested that MF patients under the care of some community hematologists may not be discussing all options including stem cell transplant, at optimal timing for their best outcomes of the disease. We urge people to try the Stem Cell Transplant Timing Tool and to discuss results with your MPN specialist. If you don’t regularly see an MPN specialist you are comfortable with, we can help you find one.

    Survival Advantage to Allogeneic Transplant in Patients with Myelofibrosis with Intermediate-1 or Higher DIPSS Score

    Predictors of Response in Patients Receiving CD34-Selected Stem Cell Infusions without Conditioning to Correct Graft Failure Following Allogeneic Stem Cell Transplantation

    Assessment of Quality of Life Following Allogeneic Stem Cell Transplant for Myelofibrosis

    High Cure Rate By Allogeneic Stem Cell Transplantation for Myelofibrosis Patients Aged 65 or Older

    As part of Patient Power’s coverage from ASH 2018, a panel of experts in MPNs, including Dr. Srdan Verstovsek, Dr. Abdulraheem Yacoub & Lindsey Lyle, as well as Andrew Schorr, gathered to discuss the latest news & developments related to research & treatment of MPNs. Click HERE to watch the video.

    For more information about the latest MPN news out of ASH, watch the video below of MPN Advocacy & Education International’s Scientific Advisor, Dr. Ruben Mesa, Director of UT Health San Antonio MD Anderson Cancer Center.