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<p>Chronic myeloid leukemia (CML) and Philadelphia (Ph)-negative myeloproliferative neoplasms (MPN) are generally distinct clonal disorders, with the co-occurrence of <i>BCR::ABL1</i> rearrangement with concomitant Ph-negative MPN rarely reported. Here we describe the largest known international cohort of Ph-negative MPN and coexisting CML providing important insights into this rare clinical scenario. We performed an international, multicenter, retrospective analysis of patients with concomitant <i>BCR::ABL1</i> rearrangement and Ph-negative MPN, identifying 61 cases from 30 centers in 7 countries, over a 29-year period (1996–2025). Thirty-one patients (50.8%) had Ph-negative MPN preceding CML, 18 patients (29.5%) had CML preceding Ph-negative MPN, and 12 patients (19.7%) had Ph-negative MPN and CML diagnosed simultaneously. We observed increased TKI resistance and myelofibrotic transformation, especially in patients initially diagnosed with Ph-negative MPN. In this group, 35.4% (<i>n</i> = 11) progressed to MF, 2 patients to blast-phase MPN, and 69.2% (<i>n</i> = 18) failed to achieve a complete cytogenetic response. The rare e1a2 <i>BCR::ABL1</i> transcript was notably prevalent which is associated with TKI resistance and a more aggressive disease course in CML. We described superior survival in those with Ph-negative MPN preceding CML, with median OS not reached, compared with 277 months for CML preceding Ph-negative MPN and 100 months for those diagnosed simultaneously (<i>p</i> = 0.05).</p>

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Myeloproliferative neoplasms with concomitant chronic myeloid leukemia are associated with TKI resistance and poor outcomes

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月27日

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  • Laura Li Gagnon,
  • Andrea Duminuco,
  • Fabio Stagno,
  • Marta Sobas,
  • Mikhail Fominykh,
  • Andres Virchis,
  • Franck-Emmanuel Nicolini,
  • Julie Hildt,
  • Helen Bentley,
  • Anna L. Godfrey,
  • Lee-Yung Shih,
  • Ashlyn Chee,
  • Adam Mead,
  • Krzysztof Lewandowski,
  • Patryk Sobieralski,
  • Guy Hannah,
  • Yasmin Reyal,
  • Thomas Cummin,
  • Jaroslaw Piszcz,
  • Kawai Yip,
  • Rebecca Frewin,
  • Srinivasan Narayanan,
  • Deborah Rahman,
  • Alero Ajayi,
  • Paul Cervi,
  • Jennifer Byrne,
  • Tom Taylor,
  • Weronika Lebowa,
  • Jiri Pavlu,
  • Siamak Arami,
  • Mansour Ceesay,
  • Dominik Chraniuk,
  • Robert Delage,
  • Annalisa Condorelli,
  • Clémence Santana,
  • Deepti H. Radia,
  • Hugues de Lavallade,
  • Dragana Milojkovic,
  • Claire Harrison,
  • Patrick Harrington

摘要

Chronic myeloid leukemia (CML) and Philadelphia (Ph)-negative myeloproliferative neoplasms (MPN) are generally distinct clonal disorders, with the co-occurrence of BCR::ABL1 rearrangement with concomitant Ph-negative MPN rarely reported. Here we describe the largest known international cohort of Ph-negative MPN and coexisting CML providing important insights into this rare clinical scenario. We performed an international, multicenter, retrospective analysis of patients with concomitant BCR::ABL1 rearrangement and Ph-negative MPN, identifying 61 cases from 30 centers in 7 countries, over a 29-year period (1996–2025). Thirty-one patients (50.8%) had Ph-negative MPN preceding CML, 18 patients (29.5%) had CML preceding Ph-negative MPN, and 12 patients (19.7%) had Ph-negative MPN and CML diagnosed simultaneously. We observed increased TKI resistance and myelofibrotic transformation, especially in patients initially diagnosed with Ph-negative MPN. In this group, 35.4% (n = 11) progressed to MF, 2 patients to blast-phase MPN, and 69.2% (n = 18) failed to achieve a complete cytogenetic response. The rare e1a2 BCR::ABL1 transcript was notably prevalent which is associated with TKI resistance and a more aggressive disease course in CML. We described superior survival in those with Ph-negative MPN preceding CML, with median OS not reached, compared with 277 months for CML preceding Ph-negative MPN and 100 months for those diagnosed simultaneously (p = 0.05).