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Understanding and treating myelofibrosis
Dec 8, 2024, 06:21

Understanding and treating myelofibrosis

Nico Gagelmann shared a post on X:

Understanding and treating myelofibrosis

A short thread on selected clinical abstracts.

Navtemadlin for JAK inhibitor refractory myelofibrosis (BOREAS):

  • reduced CD34 + counts by -82 percent at Week 24
  • 47 percent of Navtemadlin-treated patients improved fibrosis grade at Week 24
  • more than 50 percent reductions in driver mutations in 18 percent

Disease-Modifying Activity of Navtemadlin Correlates with Clinical Responses in a Randomized, Multicenter, Global Phase 3 Study (BOREAS) in JAK-Inhibitor Relapsed/Refractory Myelofibrosis

Authors: John O. Mascarenhas, Prithviraj Bose, Hsin-An Hou, Wojciech Homenda, David Ross,  Sanjay R Mohan, Jean-Jacques Kiladjian, Haifa Kathrin Al-Ali, Andrew Charles Perkins, Yulia Khalina, Tracy Clevenger, Zhuying Huang, Jesse McGreivy, Wayne Rothbaum, Srdan Verstovsek, Alessandro M. Vannucchi.

Understanding and treating myelofibrosis

Understanding and treating myelofibrosis

Efficacy and Safety of Fedratinib in Patients with Myelofibrosis and Low Baseline Platelet Counts (FREEDOM2)

  • outperformed BAT in low and high baseline platelet groups for SVR35 (low: 47 percent vs 0 percent; high: 35 percent vs 10 percent)
  • platelet increases notable early (low: +43 percent vs +11 percent; high: +19 percent vs -4.5 percent)

Authors: Haifa Kathrin Al-Ali, Claire Harrison, Ruben Mesa, Moshe Talpaz, Blanca Xicoy,  Francesco Passamonti,  Alessandro Vannucchi, Patrick Brown, Christopher Hernandez, Jia Wang, Jun He, Jean-Jacques Kiladjian.

Understanding and treating myelofibrosis

Nuvisertib (TP-3654), a PIM1 kinase inhibitor:

  • 31 percent  SVR25, and 50 percent TSS50 response
  • Significant reductions in bone pain, fatigue, night sweats
  • 33 percent improved Hemoglobin ≥1 g/dL; 32 percent platlets recovery in low baseline group
  • common Grade 1/2 AEs included nausea and diarrhea

Nuvisertib (TP-3654), an Investigational Selective PIM1 Kinase Inhibitor, Showed Durable Clinical Response and Sustained Hematological Improvement in Relapsed/Refractory Myelofibrosis Patients

Authors: Firas El Chaer, Lindsay A.M. Rein, Junichiro Yuda, Kazuya Shimoda, Akiyoshi Takami,  Michiko Ichii, James McCloskey, Joseph M. Scandura, Alessandra Iurlo, Prithviraj Bose, Tamanna Haque, Alessandro Lucchesi, Shuichi Shirane, Giulia Benevolo, Idoroenyi Amanam, Jean-Jacques Kiladjian, Pankit Vachhani, Srinivas K Tantravahi, Yasushi Onishi, Ciro Rinaldi,  Marcello Rotta, Nikki Granacher, Anand Ashwin Patel, Michael Loschi, Samah Alimam, Terrence Bradley, Stanley Cheung, Vincent Ribrag,  Sujan Kabir, Karen Ansaldo, Masataka Seki, Vincent Loksa, Zhonggai Li, Jason M. Foulks,  Jatin Shah, Raajit Rampal.

Understanding and treating myelofibrosis

TP53 in MPN, context matters:

  • Best survival: chronic phase without multihit TP53 or complex karyotype (55 months)
  • Intermediate: chronic phase + multihit TP53 or complex karyotype
  • Worst: MPN-BP/AP with or without multihit TP53 or complex karyotype (4 months)

TP53 Mutations in Myeloproliferative Neoplasms: Context-Dependent Evaluation of Prognostic Relevance

Authors: Ayalew Tefferi, Maymona Abdelmagid, Giuseppe Gaetano Loscocco, Kebede Begna, Aref Al-Kali, James M. Foran,  Jeanne Palmer, Talha Badar, Mrinal M. Patnaik, Kaaren K. Reichard, Rong He, Cinthya Zepeda Mendoza, Mithun V Shah,  Attilio Orazi, Daniel A. Arber, Animesh D. Pardanani, Alessandro M. Vannucchi, Devendra Hiwase, Naseema Gangat, Paola Guglielmelli.

Understanding and treating myelofibrosis

Personalized Transplant Decision Making for Myelofibrosis:

  • Early HCT (within 1-3 years): for high/intermediate DIPSS, MIPSS70, MYSEC-PM, and TP53
  • Delayed HCT: Favored in lower-risk cases or with ASXL1 mutations only in SMF
  • response to JAKi influenced timing

Personalized Transplant Decision Making for Myelofibrosis in the Era of Molecular Genetics and JAK Inhibition

Authors: Nico Gagelmann, Barbara Mora, Filippo Branzanti, Alessandro Maria Vannucchi, Massimo Breccia, Giuseppe Alberto Maria Palumbo, Massimiliano Bonifacio,  Erika Morsia, Elena Maria Elli, Mario Tiribelli, Giulia Benevolo, Mirko Farina, Fabrizio Pane, Roberto Massimo Lemoli, Giovanni Caocci,  Alessia Tieghi, Gianni Binotto, Francesco Cavazzini, Bruno Martino,  Alessandro Isidori, Margherita Maffioli, Elisa Rumi, Tiziano Barbui, Francisco Cervantes, Matteo Giovanni Della Porta, Andrew T. Kuykendall, Marianna Caramella, Alessandra Iurlo, Valerio De Stefano, Jean-Jacques Kiladjian, David Ross, Jason Gotlib, Timothy Devos, Marco Ruggeri, Richard T. Silver, Rachel B. Salit, Thomas Schroeder, Carmelo Gurnari, Simona Pagliuca, Christina Rautenberg, Marie Robin, Marie-Thérèse Rubio, Jaroslaw Maciejewski, Alessandro Rambaldi, Maria Chiara Finazzi, Andrea Bacigalupo,  Patrizia Chiusolo, Hans Christian Reinhardt, Bart L. Scott, Nicolaus Kröger, Francesco Passamonti, Francesca Palandri.
Understanding and treating myelofibrosis

Pulmonary hypertension in MPN:

  • associated with older age, higher VAF, larger spleens, more MF
  • more heart failure (15 percent)
  • increased hematologic risk and major adverse cardiovascular events
  • RV dysfunction (TAPSE) and high cardiac output raised risks

Prognostic Implications of Pulmonary Hypertension in Myeloproliferative Neoplasms and Predictors of Hematologic Progression

Authors: Orly Leiva, Steven Soo, Nathaniel Smilowitz, Harmony Reynolds, Binita Shah, Samuel Bernard, Michelle Hyunju Lee, Chi-Joan How, Gabriela S. Hobbs.

Understanding and treating myelofibrosis

Selinexor + Ruxolitinib:

  • 40 percent achieved more than 50 percent spleen volume reduction, 87 percent had symptom alleviation, 41 percent had more than 50 percent TSS reduction, 56 percent transfusion-dependent patients became independent
  • Common TEAEs included nausea (37 percent), anemia (37 percent), and thrombocytopenia (5 percent).

The Efficacy and Safety of Selinexor in Combination with Ruxolitinib in Ruxolitinib-Treated Myelofibrosis Patients: The Interim Analysis of a Prospective, Open-Label, Multicenter, Parallel-Cohort, Phase 2 Study

Authors: Minghui Duan, Lan Ma, Qiuling Wu, PhD, Hong Liang, Wei Wang, Lijun Mu, Hai Lin, Hebing Zhou, Hong-xia Shi, Jinghua Wang, Hongmei Jing.

Understanding and treating myelofibrosis

A Phase Ib study of CXCR4-enriched T-reg cell therapy + Ruxolitinib:

  • Symptoms: 38 percent median reduction in TSS, more than 50 percent reduction in 4/9 patients
  • 4/6 patients had spleen volume reduction, 1 with more than 35 percent
  • Reduced transfusion dependency in two

A Phase Ib, Open-Label Study of Add on Therapy with CK0804 in Participants with Myelofibrosis and Suboptimal Response to Ruxolitinib

Authors: Lucia Masarova,  Meixian Huang, Swati Goel, Sharon Bledsoe, Naveen Pemmaraju, Tapan M. Kadia,  Prithviraj Bose,  Jo Ishizawa, Guillermo Montalban-Bravo, Mi-Ae Lyu, Tara Sadeghi, Simrit Parmar, Christopher R. Flowers, Hagop M. Kantarjian.

Understanding and treating myelofibrosis

Nico Gagelmann is a physician and scientist who co-founded and co-chairs the European Society for Blood and Marrow Transplantation (EBMT) Trainee Committee, and he also serves as the chair of the EBMT subcommittee focused on CAR-T cell therapies for plasma cell disorders. His work is particularly impactful in the realm of CAR-T treatments for multiple myeloma, where he has contributed to advancing research and clinical approaches.

Akiyoshi Takami Alessandra Iurlo Alessandro Isidori Alessandro Lucchesi Alessandro M. Vannucchi Alessandro Maria Vannucchi Alessandro Rambaldi Alessandro Vannucchi Alessia Tieghi Anand Ashwin Patel Andrea Bacigalupo Andrew Charles Perkins Andrew T. Kuykendall anemia Animesh D. Pardanani Aref Al-Kali ASXL1 mutations Attilio Orazi Ayalew Tefferi Barbara Mora Bart L Scott BAT Binita Shah Blanca Xicoy Bruno Martino cancer Carmelo Gurnari CD34+ Chi-Joan How Christina Rautenberg Christopher Hernandez Christopher R. Flowers Cinthya Zepeda Mendoza Ciro Rinaldi Claire Harrison Daniel A. Arber David Ross Devendra Hiwase DIPSS Elena Maria Elli Elisa Rumi Erika Morsia Fabrizio Pane Filippo Branzanti Firas El Chaer Francesca Palandri Francesco Cavazzini Francesco Passamonti Francisco Cervantes Gabriela S Hobbs Gianni Binotto Giovanni Caocci Giulia Benevolo Giuseppe Alberto Maria Palumbo Giuseppe Gaetano Loscocco Guillermo Montalban-Bravo Hagop M. Kantarjian Haifa Kathrin Al-Ali Hans Christian Reinhardt Harmony Reynolds HCT Hsin-An Hou Idoroenyi Amanam JAK inhibitor James M Foran James McCloskey Jaroslaw Maciejewski Jason Gotlib Jason M. Foulks Jatin Shah Jean-Jacques Kiladjian Jeanne Palmer Jesse McGreivy Jia Wang Jo Ishizawa John O. Mascarenhas Joseph M. Scandura Jun He Junichiro Yuda Kaaren K. Reichard Karen Ansaldo Kazuya Shimoda Kebede Begna Lindsay A.M. Rein Lucia Masarova Marcello Rotta Marco Ruggeri Margherita Maffioli Maria Chiara Finazzi Marianna Caramella Marie Robin Marie-Therese Rubio Mario Tiribelli Masataka Seki Massimiliano Bonifacio Massimo Breccia Matteo Giovanni Della Porta Maymona Abdelmagid Meixian Huang Mi-Ae Lyu Michael Loschi Michelle Hyunju Lee Michiko Ichii MIPSS70 Mirko Farina Mithun V Shah Moshe Talpaz MPN Mrinal M Patnaik myelofibrosis MYSEC-PM Naseema Gangat Nathaniel Smilowitz nausea Naveen Pemmaraju Navtemadlin Nico Gagelmann Nicolaus Kröger Nikki Granacher Nuvisertib OncoDaily Oncology Orly Leiva Pankit Vachhani Paola Guglielmelli Patrick Brown Patrizia Chiusolo PIM1 Prithviraj Bose Pulmonary Hypertension Raajit Rampal Rachel B Salit Richard T. Silver Roberto Massimo Lemoli Rong He Ruben Mesa ruxolitinib Samah Alimam Samuel Bernard Sanjay R. Mohan selinexor Sharon Bledsoe Shuichi Shirane Simona Pagliuca Simrit Parmar Srdan Verstovsek Srinivas K Tantravahi Stanley Cheung Steven Soo Sujan Kabir SVR25 SVR35w24 Swati Goel Talha Badar Tamanna Haque Tapan M. Kadia TAPSE Tara Sadeghi TEAE Terrence Bradley Thomas Schroeder Thrombocytopenia Timothy Devos Tiziano Barbui TP53 Tracy Clevenger TSS50 VAF Valerio De Stefano Vincent Loksa Vincent Ribrag Wayne Rothbaum Wojciech Homenda Yasushi Onishi Yulia Khalina Zhonggai Li Zhuying Huang