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Signal Inhibitors in Waldenstrom’s Macroglobulinemia

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Waldenström’s Macroglobulinemia

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Abstract

Whole genome sequencing has identified actionable mutations in MYD88 and CXCR4 in lymphoplasmacytic cells from Waldenstrom’s Macroglobulinemia (WM) patients that promote multiple survival cascades that include Bruton’s Tyrosine Kinase (BTK), PI3Kδ/AKT, and ERK1/2. Transcriptome studies have also identified aberrations in MYD88, CXCR4, and BCL2 survival signaling. Novel treatment options for WM include inhibitors that target BTK, PI3Kδ/AKT, CXCR4, and BCL2. Ibrutinib, an inhibitor of BTK, has shown high rates of activity and durable responses in relapsed/refractory WM patients. Both MYD88 and CXCR4 tumor mutation status impact ibrutinib response.

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Author information

Authors and Affiliations

  1. Bing Center for Waldenström’s Macroglobulinemia, Dana Farber Cancer Institute, Boston, MA, USA

    Steven P. Treon MD, PhD, Guang Yang PhD, Zachary R. Hunter PhD & Jorge J. Castillo MD

  2. Harvard Medical School, Boston, MA, USA

    Steven P. Treon MD, PhD, Guang Yang PhD, Zachary R. Hunter PhD & Jorge J. Castillo MD

Authors
  1. Steven P. Treon MD, PhD
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  2. Guang Yang PhD
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  3. Zachary R. Hunter PhD
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  4. Jorge J. Castillo MD
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Corresponding author

Correspondence to Steven P. Treon MD, PhD .

Editor information

Editors and Affiliations

  1. Haematology, Hôpital Pitié-Salpêtriêre, Paris, France

    Véronique Leblond

  2. Dana Farber Cancer Institute, Boston, Massachusetts, USA

    Steve Treon

  3. School of Medicine, University of Athens, Athens, Greece

    Meletios Dimoploulos

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© 2017 Springer International Publishing Switzerland

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Treon, S.P., Yang, G., Hunter, Z.R., Castillo, J.J. (2017). Signal Inhibitors in Waldenstrom’s Macroglobulinemia. In: Leblond, V., Treon, S., Dimoploulos, M. (eds) Waldenström’s Macroglobulinemia. Springer, Cham. https://doi.org/10.1007/978-3-319-22584-5_22

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  • DOI: https://doi.org/10.1007/978-3-319-22584-5_22

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-22583-8

  • Online ISBN: 978-3-319-22584-5

  • eBook Packages: MedicineMedicine (R0)

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