Skip to main content

Advertisement

SpringerLink
Log in

Long-term efficacy and safety profile of splenectomy for pediatric chronic immune thrombocytopenia

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

Abstract

There are few reports of the long-term efficacy of splenectomy in children with immune thrombocytopenia (ITP). In a 33-year period, we performed splenectomies in 23 pediatric patients with ITP at a single institution in Japan. The age at surgery was 5–22 years with a median of 10 years. The follow-up period was 1–141 months with a median of 48 months. Before surgery, we confirmed the presence or absence of the accessory spleen by contrast-enhanced CT scan and we recommended vaccination with pneumococcal vaccine. Four patients underwent laparotomy before 1998, and 19 patients underwent laparoscopic surgery after 1999. Splenectomy showed high efficacy with a partial response rate of 83% and a complete response rate of 74%. Complete response was maintained in 70% of patients until the end of the observation period, and 91% were able to discontinue long-term management drugs such as steroids. No serious complications such as infectious diseases were observed. Although the number of cases here was small, the long-term efficacy and safety of splenectomy makes it a viable option in pediatric ITP despite the existence of newer therapeutic agents. Further research is necessary to compare the long-term efficacy and safety of splenectomy with new therapeutic agents.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Data availability statement

The data that support the findings of this study are available from the corresponding author, K.I., upon reasonable request.

References

  1. Finianos A, Mujadzic H, Peluso H, Mujadzic T, Taher A, Abougergi MS. Temporal trends and outcome of splenectomy in adults with immune thrombocytopenia in the USA. Ann Hematol. 2021;100(4):941–52.

    Article  PubMed  Google Scholar 

  2. Kühne T, Blanchette V, Buchanan G, Ramenghi U, Donato H, Tamminga R. Splenectomy in children with idiopathic thrombocytopenic purpura: a prospective study of 134 children from the intercontinental childhood ITP study group. Pediatr Blood Cancer. 2007;49(6):829–34.

    Article  PubMed  Google Scholar 

  3. Kubota M, Adachi S, Usami I, Okada M, Kitoh T, Shiota M, et al. Characterization of chronic idiopathic thrombocytopenic purpura in Japanese children: a retrospective multi-center study. Int J Hematol. 2010;91(2):252–7.

    Article  PubMed  Google Scholar 

  4. Kurata Y, Fujimura K, Kuwana M, Tomiyama Y, Murata M. Epidemiology of primary immune thrombocytopenia in children and adults in Japan: a population-based study and literature review. Int J Hematol. 2011;93(3):329–35.

    Article  PubMed  Google Scholar 

  5. Ito M, Yagasaki H, Kanezawa K, Shimozawa K, Hirai M, Morioka I. Incidence and outcomes of refractory immune thrombocytopenic purpura in children: a retrospective study in a single institution. Sci Rep. 2021;11(1):1–6. https://doi.org/10.1038/s41598-021-93646-2.

    Article  CAS  Google Scholar 

  6. Miura T, Mimaya J, Ishii E, Fujisawa K, Ohta S, Taki M, et al. A Nationwide survey of childhood idiopathic thrombocytopenic purpura in 2006. Jpn J Pediatr Hematol. 2008;22:378–81 ((in Japanese)).

    Google Scholar 

  7. Kato M, Koh K, Kikuchi A, Hanada R. Spontaneous improvement of chronic immune thrombocytopenia in children: experience of 56 patients at a single institute. Int J Hematol. 2012;96(6):729–32.

    Article  PubMed  Google Scholar 

  8. Ben-Yehuda D, Gillis S, Eldor A. Clinical and therapeutic experience in 712 Israeli patients with idiopathic thrombocytopenic purpura. Acta Haematol. 1994;91(1):1–6.

    Article  CAS  PubMed  Google Scholar 

  9. Meekes I, Van der Staak F, Van Oostrom C. Results of splenectomy performed on a group of 91 children. Eur J Pediatr Surg. 1996;6(1):19–22.

    Article  Google Scholar 

  10. Mantadakis E, Buchanan GR. Elective splenectomy in children with idiopathic thrombocytopenic purpura. Am J Pediatr Hematol Oncol. 2000;22(2):148–53.

    Article  CAS  Google Scholar 

  11. Hemmila MR, Foley DS, Castle VP, Hirschl RB. The response to splenectomy in pediatric patients with idiopathic thrombocytopenic purpura who fail high-dose intravenous immune globulin. J Pediatr Surg. 2000;35(6):967–72.

    Article  CAS  PubMed  Google Scholar 

  12. Bussel JB, Kaufmann CP, Ware RE, Woloski BMR. Do the acute platelet responses of patients with immune thrombocytopenic purpura (ITP) to IV anti-D and to IV gammaglobulin predict response to subsequent splenectomy? Am J Hematol. 2001;67(1):27–33.

    Article  CAS  PubMed  Google Scholar 

  13. Lowe EJ, Buchanan GR. Idiopathic thrombocytopenic purpura diagnosed during the second decade of life. J Pediatr. 2002;141(2):253–8.

    Article  PubMed  Google Scholar 

  14. El-Alfy MS, El-Tawil MM, Shahein N. 5- to 16-year follow-up following splenectomy in chronic immune thrombocytopenic purpura in children. Acta Haematol. 2003;110(1):20–4.

    Article  PubMed  Google Scholar 

  15. Bourgeois E, Caulier MT, Delarozee C, Brouillard M, Bauters F, Fenaux P. Long-term follow-up of chronic autoimmune thrombocytopenic purpura refractory to splenectomy: a prospective analysis. Br J Haematol. 2003;120(6):1079–88.

    Article  PubMed  Google Scholar 

  16. Aronis S, Platokouki H, Avgeri M, Pergantou H, Keramidas D. Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children. Acta Paediatr. 2004;93(5):638–42.

    Article  CAS  PubMed  Google Scholar 

  17. Watts RG. Idiopathic thrombocytopenic purpura: a 10-year natural history study at the childrens hospital of Alabama. Clin Pediatr. 2004;43(8):691–702.

    Article  Google Scholar 

  18. Jayabose S, Levendoglu-Tugal O, Ozkaynkak MF, Visintainer P, Sandoval C. Long-term outcome of chronic idiopathic thrombocytopenic purpura in children. J Pediatr Hematol Oncol. 2004;26(11):724–6.

    Article  PubMed  Google Scholar 

  19. Kwon HC, Chang HM, Young RC, Min CK, Kyeong HK, Jin YH, et al. Prognostic factors of response to laparoscopic splenectomy in patients with idiopathic thrombocytopenic purpura. J Korean Med Sci. 2005;20(3):417–20.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Wang T, Xu M, Ji L, Yang R. Splenectomy for chronic idiopathic thrombocytopenic purpura in children: a single center study in China. Acta Haematol. 2006;115(1–2):39–45.

    Article  PubMed  Google Scholar 

  21. Ramenghi U, Amendola G, Farinasso L, Giordano P, Loffredo G, Nobili B, et al. Splenectomy in children with chronic ITP: long-term efficacy and relation between its outcome and responses to previous treatments. Pediatr Blood Cancer. 2006;47(S5):742–5. https://doi.org/10.1002/pbc.20978.

    Article  PubMed  Google Scholar 

  22. Donato H, Picón A, Rapetti MC, Rosso A, Schvartzman G, Drozdowski C, et al. Splenectomy and spontaneous remission in children with chronic idiopathic thrombocytopenic purpura. Pediatr Blood Cancer. 2006;47(S5):737–9. https://doi.org/10.1002/pbc.20982.

    Article  PubMed  Google Scholar 

  23. Machado NO, Grant CS, Alkindi S, Daar S, Al-Kindy N, Al Lamki Z, et al. Splenectomy for haematological disorders: a single center study in 150 patients from Oman. Int J Surg. 2009;7(5):476–81. https://doi.org/10.1016/j.ijsu.2009.08.004.

    Article  PubMed  Google Scholar 

  24. Bansal D, Bhamare TA, Trehan A, Ahluwalia J, Varma N, Marwaha RK. Outcome of chronic idiopathic thrombocytopenic purpura in children. Pediatr Blood Cancer. 2010;2010(54):1388–9.

    Google Scholar 

  25. Hollander LL, Leys CM, Weil BR, Rescorla FJ. Predictive value of response to steroid therapy on response to splenectomy in children with immune thrombocytopenic purpura. Surgery. 2011;150(4):643–8. https://doi.org/10.1016/j.surg.2011.07.063.

    Article  PubMed  Google Scholar 

  26. Rosthøj S, Rajantie J, Treutiger I, Zeller B, Tedgård U, Henter JI. Duration and morbidity of chronic immune thrombocytopenic purpura in children: five-year follow-up of a Nordic cohort. Acta Paediatr Int J Paediatr. 2012;101(7):761–6.

    Article  Google Scholar 

  27. Evim MS, Baytan B, Güneş AM. Childhood immune thrombocytopenia: long-term follow-up data evaluated by the criteria of the international working group on immune thrombocytopenic purpura. Turkish J Hematol. 2014;31(1):32–9.

    Article  Google Scholar 

  28. Ay Y, Karapinar TH, Oymak Y, Toret E, Demirag B, Ince D, et al. Retrospective analysis of rituximab therapy and splenectomy in childhood chronic and refractory immune thrombocytopenic purpura. Blood Coagul Fibrinol. 2016;27(4):431–5.

    Article  CAS  Google Scholar 

  29. Ahmed R, Devasia AJ, Viswabandya A, Lakshmi KM, Abraham A, Karl S, et al. Long-term outcome following splenectomy for chronic and persistent immune thrombocytopenia (ITP) in adults and children: splenectomy in ITP. Ann Hematol. 2016;95(9):1429–34. https://doi.org/10.1007/s00277-016-2738-3.

    Article  CAS  PubMed  Google Scholar 

  30. Palandri F, Polverelli N, Sollazzo D, Romano M, Catani L, Cavo M, et al. Have splenectomy rate and main outcomes of ITP changed after the introduction of new treatments? A monocentric study in the outpatient setting during 35 years. Am J Hematol. 2016;91(4):E267–72.

    Article  CAS  PubMed  Google Scholar 

  31. Ducassou S, Gourdonneau A, Fernandes H, Leverger G, Pasquet M, Fouyssac F, et al. Second-line treatment trends and long-term outcomes of 392 children with chronic immune thrombocytopenic purpura: the French experience over the past 25 years. Br J Haematol. 2020;189(5):931–42.

    Article  CAS  PubMed  Google Scholar 

  32. Gwilliam NR, Lazar DA, Brandt ML, Mahoney DH, Wesson DE, Mazziotti MV, et al. An analysis of outcomes and treatment costs for children undergoing splenectomy for chronic immune thrombocytopenia purpura. J Pediatr Surg. 2012;47(8):1537–41. https://doi.org/10.1016/j.jpedsurg.2012.02.013.

    Article  PubMed  Google Scholar 

  33. Kojouri K, Vesely SK, Terrell DR, George JN. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications. Blood. 2004;104(9):2623–34.

    Article  CAS  PubMed  Google Scholar 

  34. Facon T, Caulier M, Fenaux P. Accessory spleen in recurrent chronic immune thrombocytopenic purpura. Am J Hematol. 1992;41:184–9.

    Article  CAS  PubMed  Google Scholar 

  35. Ruggeri M, Tosetto A, Palandri F, Polverelli N, Mazzucconi MG, Santoro C, et al. Thrombotic risk in patients with primary immune thrombocytopenia is only mildly increased and explained by personal and treatment-related risk factors. J Thromb Haemost. 2014;12(8):1266–73.

    Article  CAS  PubMed  Google Scholar 

  36. Cines DB, Wasser J, Rodeghiero F, Chong BH, Steurer M, Provan D, et al. Safety and efficacy of romiplostim in splenectomized and nonsplenectomized patients with primary immune thrombocytopenia. Haematologica. 2017;102(8):1342–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Neunert C, Terrell DR, Arnold DM, Buchanan G, Cines DB, Cooper N, et al. American society of hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019;3(23):3829–66.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

M.M and K.K. designed the research; M.M., T.I., T.I., M.H., R.K., Y.M., H.K., K.F., K.O., Y.A., and K.K. collected data; K.I. analyzed data; K.I., M.M., and K.K. drafted the paper; all authors discussed the results and commented on the manuscript.

Author information

Authors and Affiliations

  1. Department of Hematology/Oncology, Saitama Children’s Medical Center, 1-2 Shintoshin, Chuou-Ku, Saitama, 330-8777, Japan

    Kyohei Isshiki, Makiko Mori, Tomoya Irikura, Takahiro Ishikawa, Mamoru Honda, Ryota Kaneko, Yuichi Mitani, Hirohito Kubota, Kohei Fukuoka, Koichi Oshima, Yuki Arakawa & Katsuyoshi Koh

  2. Department of Pediatrics, Saitama City Hospital, 2460 Mimuro, Midori-Ku, Saitama, 336-8522, Japan

    Kyohei Isshiki

Authors
  1. Kyohei Isshiki
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Makiko Mori
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Tomoya Irikura
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Takahiro Ishikawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mamoru Honda
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ryota Kaneko
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Yuichi Mitani
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Hirohito Kubota
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Kohei Fukuoka
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Koichi Oshima
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Yuki Arakawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Katsuyoshi Koh
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Kyohei Isshiki.

Ethics declarations

Conflicts of interest

The authors declare that they have no conflict of interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Isshiki, K., Mori, M., Irikura, T. et al. Long-term efficacy and safety profile of splenectomy for pediatric chronic immune thrombocytopenia. Int J Hematol 117, 774–780 (2023). https://doi.org/10.1007/s12185-022-03529-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-022-03529-z

Keywords

Search

Navigation