Skip to main content
Log in

Relapsed APL patient with variant NPMRARα fusion responded to arsenic trioxide-based therapy and achieved long-term survival

  • Case Report
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

The t(5;17)/NPMRARα is the second variant chromosomal translocation in acute promyelocytic leukemia (APL) to be characterized and also the second most plentiful variant translocation. So far, there is a lack of information on the effectiveness of arsenic trioxide (ATO) in relapsed APL with variant RARα chimera including t(5;17)/NPMRARα. We report here a long-term survived APL patient with variant NPMRARα fusion who relapsed four times and each time responded well to ATO or ATO-based re-induction therapy. The patient had received a total of more than 3,500 mg of ATO, but showed no obvious arsenic-related toxicities. This case illustrates the long-term efficiency and safety of ATO-based therapy not only in newly diagnosed APL, but also in relapsed APL including those with variant translocations.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Sirulnik A, Melnick A, Zelent A, Licht JD. Molecular pathogenesis of acute promyelocytic leukemia and APL variants. Best Pract Res Clin Haematol. 2003;16:387–408.

    Article  CAS  PubMed  Google Scholar 

  2. Wang ZY, Chen Z. Acute promyelocytic leukemia: from highly fatal to highly curable. Blood. 2008;111:2505–14.

    Article  CAS  PubMed  Google Scholar 

  3. Catalano A, Dawson MA, Somana K, Opat S, Schwarer A, Campbell LJ, et al. The PRKAR1A gene is fused to RARA in a new variant acute promyelocytic leukemia. Blood. 2009;110:4073–6.

    Article  Google Scholar 

  4. Sanz MA, Grimwade D, Tallman MS, Lowenburg B, Fenaux P, Estey EH, et al. Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet. Blood. 2009;113:1875–91.

    Article  CAS  PubMed  Google Scholar 

  5. Sun HD, Ma L, Hu XC, Zhang TD. Ai-Lin I treated 32 cases of acute promyelocytic leukemia. Chin J Integr Chin West Med. 1992;12:170–2.

    Google Scholar 

  6. Mathews V, Balasubramanian P, Shaji RV, George B, Chandy M, Srivastava A. Arsenic trioxide in the treatment of newly diagnosed acute promyelocytic leukemia: a single center experience. Am J Hematol. 2002;70:292–9.

    Article  CAS  PubMed  Google Scholar 

  7. Mathews V, George B, Lakshimi KM, Viswabandya A, Bajel A, Balasubramanian P, et al. Single-agent arsenic trioxide in the treatment of newly diagnosed acute promyelocytic leukemia: durable remissions with minimal toxicity. Blood. 2006;107:2627–32.

    Article  CAS  PubMed  Google Scholar 

  8. Shen ZX, Chen GQ, Ni JH, Li XS, Xiong SM, Qiu QY, et al. Use of arsenic trioxide (As2O3) in the treatment of acute promyelocytic leukemia (APL): II, clinical efficacy and pharmacokinetics in relapsed patients. Blood. 1997;89:3354–60.

    CAS  PubMed  Google Scholar 

  9. Soignet SL, Frankel SR, Douer D, Tallman MS, Kantarjian H, Calleja E, et al. United States multicenter study of arsenic trioxide in relapsed acute promyelocytic leukemia. J Clin Oncol. 2001;19:3852–60.

    CAS  PubMed  Google Scholar 

  10. Hu J, Liu YF, Wu CF, Xu F, Shen ZX, Zhu YM, et al. Long-term efficacy and safety of all-trans retinoic acid/arsenic trioxide-based therapy in newly diagnosed acute promyelocytic leukemia. Proc Natl Acad Sci USA. 2009;106:3342–7.

    Article  CAS  PubMed  Google Scholar 

  11. Koken MHM, Daniel MT, Gianni M, Zelent A, Licht J, Buzyn A, et al. Retinoic acid, but not arsenic trioxide, degrades the PLZF/RARalpha fusion protein, without inducing terminal differentiation or apoptosis, in a RA-therapy resistant t(11;17)(q23;q21) APL patient. Oncogene. 1999;18:1113–8.

    Article  CAS  PubMed  Google Scholar 

  12. Redner RL. Variations on a theme: the alternate translocations in APL. Leukemia. 2002;16:1927–32.

    Article  CAS  PubMed  Google Scholar 

  13. Okazuka K, Masuko M, Seki Y, Hama H, Honma N, Furukawa T, et al. Successful all-trans retinoic acid treatment of acute promyelocytic leukemia in a patient with NPM-RAR fusion. Int J Hematol. 2007;86:246–9.

    Article  CAS  PubMed  Google Scholar 

  14. Qiu HR, Li JY, Miao KR, Wang R, Zhang JF, Zhang W. A case of acute promyelocytic leukemia with variant t(5;17) and trisomy 22. Chin J Med Genet. 2008;25:430–3.

    Google Scholar 

Download references

Acknowledgments

I would like to thank Drs Chin-Hon Pui and GCF Chan for careful reading of the manuscript. This work was supported in part by the National Natural Science Foundation of China (30670895).

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Zhigui Ma.

About this article

Cite this article

Chen, Y., Gu, L., Zhou, C. et al. Relapsed APL patient with variant NPMRARα fusion responded to arsenic trioxide-based therapy and achieved long-term survival. Int J Hematol 91, 708–710 (2010). https://doi.org/10.1007/s12185-010-0553-5

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-010-0553-5

Keywords

Navigation