Advertisement

International Journal of Hematology

, Volume 107, Issue 1, pp 64–68 | Cite as

Modified immunosuppressive therapy with porcine antilymphocyte globulin plus delayed cyclosporine A in children with severe aplastic anemia

  • Qingya Cui
  • Pingping Sha
  • Haifei Chen
  • Hongshi Shen
  • Longmei Qin
  • Zhengyang Li
  • Tianqin Wu
  • Zhaoyue Wang
Original Article
  • 140 Downloads

Abstract

Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine (CsA) is the standard treatment for children with severe aplastic anemia (SAA) with no human leukocyte antigen-matched siblings. Due to the unavailability of horse ATG in China, porcine antilymphocyte globulin (p-ALG), which is less expensive and more effective than rabbit ATG, is widely used. We sought to evaluate the efficacy and safety profile of modified IST with p-ALG plus delayed CsA at day 21 in 50 SAA children. Eighteen SAA patients who progressed from nonsevere aplastic anemia (NSAA) were classified as SAA-II; the other 32 patients were classified as SAA-I. Overall response (OR) rates at 3, 6 and 12 months were 56, 64 and 62%, respectively. The 10-year overall survival (OS) rate and disease-free survival (DFS) rate were 80 and 56%. The OR, OS and DFS rates in the SAA-I group were clearly better than those in the SAA-II group. Death rate from infection within 30 days was 4%. Modified IST with p-ALG plus delayed CsA is a reliable and well-tolerated treatment for children with SAA, and reduces early death due to infection. Modified IST is more suitable for children with SAA-I.

Keywords

Severe aplastic anemia Porcine antilymphocyte globulin Modified immunosuppressive therapy Infection 

Notes

Acknowledgements

This work was supported by the Priority Academic Program Development of Jiangsu Higher Education Institutions (PAPD), Jiangsu Province’s Key Medical Center (ZX201102), and Life and health special funds of the Jiangsu Province’s Science and Technology Bureau (BL2012005).

Compliance with ethical standards

Conflict of interest

All authors declare no conflicts of interest.

References

  1. 1.
    Brodsky RA, Jones RJ. Aplastic anemia. Lancet. 2005;365(9471):1647–56.CrossRefPubMedGoogle Scholar
  2. 2.
    Bacigalupo A, Socie G, Schrezenmeier H, Tichelli A, Locasciulli A, Fuehrer M, et al. Bone marrow versus peripheral blood as the stem cell source for sibling transplants in acquired aplastic anemia: survival advantage for bone marrow in all age groups. Haematologica. 2012;97(8):1142–8.Google Scholar
  3. 3.
    Ades L, Mary JY, Robin M, Ferry C, Porcher R, Esperou H, et al. Long-term outcome after bone marrow transplantation for severe aplastic anemia. Blood. 2004;103(7):2490–7.CrossRefPubMedGoogle Scholar
  4. 4.
    Frickhofen N, Rosenfeld SJ. Immunosuppressive treatment of aplastic anemia with antithymocyte globulin and cyclosporine. Semin Hematol. 2000;37(1):56–68.CrossRefPubMedGoogle Scholar
  5. 5.
    Bing H, Siyi Y, Wei Z, Jian L, Minghui D, Li J, et al. The use of anti-human T lymphocyte porcine immunoglobulin and cyclosporine a to treat patients with acquired severe aplastic anemia. Acta Haematol. 2010;124(4):245–50.CrossRefPubMedGoogle Scholar
  6. 6.
    Chen M, Liu C, Zhuang J, Zou N, Xu Y. Zhang We t al. Long-term follow-up study of porcine anti-human thymocyte immunoglobulin therapy combined with cyclosporine for severe aplastic anemia. Eur J Haematol. 2016;96(3):291–6.CrossRefPubMedGoogle Scholar
  7. 7.
    Wei J, Huang Z, Guo J, Zhang Y, Wang C, Zhu X, et al. Porcine antilymphocyte globulin (p-ALG) plus cyclosporine A (CsA) treatment in acquired severe aplastic anemia: a retrospective multicenter analysis. Ann Hematol. 2015;94(6):955–62.CrossRefPubMedGoogle Scholar
  8. 8.
    Liu L, Ding L, Hao L, Zhang X, Li X, Zhang L, et al. Efficacy of porcine antihuman lymphocyte immunoglobulin compared to rabbit antithymocyte immunoglobulin as a first-line treatment against acquired severe aplastic anemia. Ann Hematol. 2015;94(5):729–37.CrossRefPubMedGoogle Scholar
  9. 9.
    Ma X, Wang J, Zhang W, Cao X, Chen Y, He A, et al. Comparison of porcine anti-human lymphocyte globulin and rabbit anti-human thymocyte globulin in the treatment of severe aplastic anemia: a retrospective single-center study. Eur J Haematol. 2016;96(3):260–8.CrossRefPubMedGoogle Scholar
  10. 10.
    Zhang X, Liu L, Zhao G, Si Y, Lin G, Ma A, et al. Porcine anti-human lymphocyte immunoglobulin plus cydosporine in the treatment of severe aplastic anemia. Zhonghua Xue Ye Xue Za Zhi. 2014;35(5):451–3.PubMedGoogle Scholar
  11. 11.
    Han B, Yan SY, Zou N, Zhang W, Li J, Duan MH, et al. Porcine anti-human lymphocyte globulin plus cyclosporine A therapy for severe aplastic anemia. Zhonghua Xue Ye Xue Za Zhi. 2011;32(4):241–4.PubMedGoogle Scholar
  12. 12.
    Locasciulli A, van’t Veer L, Bacigalupo A, Hows J, Van Lint MT, Gluckman E, et al. Treatment with marrow transplantation or immunosuppression of childhood acquired severe aplastic anemia: a report from the EBMT SAA working party. Bone Marrow Transpl. 1990;6(3):211–7.Google Scholar
  13. 13.
    Yoshida N, Yagasaki H, Hama A, Takahashi Y, Kosaka Y, Kobayashi R, et al. Predicting response to immunosuppressive therapy in childhood aplastic anemia. Haematologica. 2011;96(5):771–4.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Shin SH, Yoon JH, Yahng SA, Lee SE, Cho BS, Eom KS, et al. The efficacy of rabbit antithymocyte globulin with cyclosporine in comparison to horse antithymocyte globulin as a first-line treatment in adult patients with severe aplastic anemia: a single-center retrospective study. Ann Hematol. 2013;92(6):817–24.CrossRefPubMedGoogle Scholar
  15. 15.
    Rosenfeld S, Follmann D, Nunez O, Young NS. Antithymocyte globulin and cyclosporine for severe aplastic anemia: association between hematologic response and long-term outcome. JAMA. 2003;289(9):1130–5.CrossRefPubMedGoogle Scholar
  16. 16.
    Kojima S, Hibi S, Kosaka Y, Yamamoto M, Tsuchida M, Mugishima H, et al. Immunosuppressive therapy using antithymocyte globulin, cyclosporine, and danazol with or without human granulocyte colony-stimulating factor in children with acquired aplastic anemia. Blood. 2000;96(6):2049–54.PubMedGoogle Scholar
  17. 17.
    Xie X, Shi W, Zhou X, Shao Y, Qiao X. Comparison of rabbit antithymocyte globulin and Jurkat cell-reactive anti-T lymphocyte globulin as a first-line treatment for children with aplastic anemia. Exp Hematol. 2014;42(6):431–8.CrossRefPubMedGoogle Scholar
  18. 18.
    Scheinberg P, Wu CO, Nunez O, Young NS. Long-term outcome of pediatric patients with severe aplastic anemia treated with antithymocyte globulin and cyclosporine. J Pediatr. 2008;153(6):814–9.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Chen C, Xue HM, Xu HG, Li Y, Huang K, Zhou DH, et al. Rabbit-antithymocyte globulin combined with cyclosporin A as a first-line therapy: improved, effective, and safe for children with acquired severe aplastic anemia. J Cancer Res Clin Oncol. 2012;138(7):1105–11.CrossRefPubMedGoogle Scholar
  20. 20.
    Li X, Shi J, Ge M, Shao Y, Huang J, Huang Z, et al. Outcomes of optimized over standard protocol of rabbit antithymocyte globulin for severe aplastic anemia: a single-center experience. PLoS ONE. 2013;8(3):e56648.CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Scheinberg P, Young NS. How I treat acquired aplastic anemia. Blood. 2012;120(6):1185–96.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2017

Authors and Affiliations

  • Qingya Cui
    • 1
  • Pingping Sha
    • 1
  • Haifei Chen
    • 2
  • Hongshi Shen
    • 2
  • Longmei Qin
    • 2
  • Zhengyang Li
    • 2
  • Tianqin Wu
    • 2
  • Zhaoyue Wang
    • 1
  1. 1.Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow UniversityKey Lab of Thrombosis and Hemostasis of Ministry of HealthSuzhouChina
  2. 2.The Department of HematologyPLA 100th HospitalSuzhouChina

Personalised recommendations