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Development of acute lymphoblastic leukemia with IgH-EPOR in a patient with secondary erythrocytosis

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Abstract

We report the first patient to develop ALL with a fusion gene of the erythropoietin receptor (EPOR) with immunoglobulin heavy chain (IgH) 22 years after a diagnosis of secondary erythrocytosis with unknown etiology. The IgH-EPOR rearrangement is known to induce increased expression of EPOR, and activates EPO-associated signal pathways by exogenous EPO stimulation, resulting in the increased proliferation and survival of IgH-EPOR-positive leukemic cells. Interestingly, this case may provide supporting the possibility that IgH-EPOR-positive ALL has a growth advantage under sustained high concentrations of EPO.

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References

  1. Den Boer ML, van Slegtenhorst M, De Menezes RX, Cheok MH, Buijs-Gladdines JG, Peters ST, et al. A subtype of childhood acute lymphoblastic leukaemia with poor treatment outcome: a genome-wide classification study. Lancet Oncol. 2009;10:125–34.

    Article  Google Scholar 

  2. Roberts KG, Morin RD, Zhang J, Hirst M, Zhao Y, Su X, et al. Genetic alterations activating kinase and cytokine receptor signaling in high-risk acute lymphoblastic leukemia. Cancer Cell. 2012;22:153–66.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Roberts KG, Li Y, Payne-Turner D, Harvey RC, Yang YL, Pei D, et al. Targetable kinase-activating lesions in Ph-like acute lymphoblastic leukemia. N Engl J Med. 2014;371:1005–15.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Russell LJ, De Castro DG, Griffiths M, Telford N, Bernard O, Panzer-Grumayer R, et al. A novel translocation, t(14;19)(q32;p13), involving IGH@ and the cytokine receptor for erythropoietin. Leukemia. 2009;23:614–7.

    Article  CAS  PubMed  Google Scholar 

  5. Hedley BD, Allan AL, Xenocostas A. The role of erythropoietin and erythropoiesis-stimulating agents in tumor progression. Clin Cancer Res. 2011;17:6373–80.

    Article  CAS  PubMed  Google Scholar 

  6. Iacobucci I, Li Y, Roberts KG, Dobson SM, Kim JC, Payne-Turner D, et al. Truncating erythropoietin receptor rearrangements in acute lymphoblastic leukemia. Cancer Cell. 2016;29:186–200.

    Article  CAS  PubMed  Google Scholar 

  7. Kantarjian H, Thomas D, O’Brien S, Cortes J, Giles F, Jeha S, et al. Long-term follow-up results of hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD), a dose-intensive regimen, in adult acute lymphocytic leukemia. Cancer. 2004;101:2788–801.

    Article  CAS  PubMed  Google Scholar 

  8. Imamura T, Kiyokawa N, Kato M, Imai C, Okamoto Y, Yano M, et al. Characterization of pediatric Philadelphia-negative B-cell precursor acute lymphoblastic leukemia with kinase fusions in Japan. Blood Cancer J. 2016;6:e419.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Russell LJ, Enshaei A, Jones L, Erhorn A, Masic D, Bentley H, et al. IGH@ translocations are prevalent in teenagers and young adults with acute lymphoblastic leukemia and are associated with a poor outcome. J Clin Oncol. 2014;32:1453–62.

    Article  PubMed  Google Scholar 

  10. Swift S, Ellison AR, Kassner P, McCaffery I, Rossi J, Sinclair AM, et al. Absence of functional EpoR expression in human tumor cell lines. Blood. 2010;115:4254–63.

    Article  CAS  PubMed  Google Scholar 

  11. Torrano V, Procter J, Cardus P, Greaves M, Ford AM. ETV6–RUNX1 promotes survival of early B lineage progenitor cells via a dysregulated erythropoietin receptor. Blood. 2011;118:4910–8.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Kenichi Sakamoto.

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Sakamoto, K., Tanaka, S., Tomoyasu, C. et al. Development of acute lymphoblastic leukemia with IgH-EPOR in a patient with secondary erythrocytosis. Int J Hematol 104, 741–743 (2016). https://doi.org/10.1007/s12185-016-2083-2

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  • DOI: https://doi.org/10.1007/s12185-016-2083-2

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