Clinical Presentation and Therapy of Primary Immune Thrombocytopenia Resistant to Splenectomy

Keywords: Autoimmune thrombocytopenia, Refractory, idiopathic thrombocytopenic purpura, Thrombopoietin receptor agonists

Abstract

Background: A satisfactory therapeutic response is achieved with splenectomy in 60–80% of the patients diagnosed with immune thrombocytopenic purpura (ITP). There is an ongoing consensus on the short-term efficacy of splenectomy, however, its long-term efficacy remains controversial. Our aim was to establish the frequency of resistance and relapse after splenectomy, the occurrence of complications, the therapeutic strategies and the drug efficacy in splenectomy-resistant ITP.

Methods: We retrospectively analyzed 138 adult ITP patients who had been previously diagnosed and treated at the Clinic of Hematology, Clinical Center of Serbia, and who underwent splenectomy between 1987 and 2018.

Results: Of the 138 ITP patients, 20.3% (n=28/138) were refractory to splenectomy, 11.6% (n=16/138) relapsed and 8.7% (n=12/138) were primarily resistant. The average post-splenectomy follow-up period was 117 months (range 3-474). The average follow-up period of the patients resistant to splenectomy was 147 months (range 23-474). Of the patients refractory to splenectomy, 67.8% (n=19/28) showed a good therapeutic response: 49% (n=14/28) complete remission and 18.8% (n=5/28) partial remission. The response was usually achieved using the following drugs: romiplostim (100%), eltrombopag (75%), cyclosporine (66.67%), mycophenolate mofetil (50%), danazol (50%) and corticosteroids (40.9%). Hemorrhagic and non-hemorrhagic complications occurred in 78.6% (n=22/28) and 28.6% (n=8/28) of the patients, respectively.

Conclusion: Splenectomy remains a very efficient therapeutic modality for the treatment of ITP patients with a high percentage of splenectomy-resistant patients achieving remission. Thrombopoietin receptor agonists have shown exceptional results so far in the treatment of refractory ITP patients.

Author Biography

Aleksandar Kara-Jovanović, Medical Faculty, University in Belgrade, Belgrade, Serbia.

Aleksandar Kara-Jovanović is currently a 6th-year medical student in the Faculty of Medicine at the University of Belgrade, Serbia within the six-year integrated academic studies. He is currently working on a research paper concerning thrombotic events in acute myeloid leukemia.

References

Lambert MP, Gernsheimer TB. Clinical updates in adult immune thrombocytopenia. Blood. 2017 May 25;129(21):2829-35.

Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009 Mar 12;113(11):2386-93.

Portielje JE, Westendorp RG, Kluin-Nelemans HC, Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001 May 1;97(9):2549-54.

Cohen YC, Djuibegovic B, Shamai-Lubovitz O, Mozes B. The bleeding risk and natural history of idiopathic thrombocytopenic purpura in patients with persistent low platelet counts. Arch Intern Med. 2000 Jun 12;160(11):1630-8.

Kistanguri G, McCrae KR. Immune Thrombocytopenia. Hematol Oncol Clin North Am. 2013 Jun;27(3):495–520.

Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-maggs P, James B, et al. International consensus report on the investigation and management of primary immune thrombocytopenia International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010 Jan 14;115(2):168-86.

British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. Br J Haematol. 2003 Feb;120(4):574-96.

Chaturvedi S, Arnold DM, McCrae KR. Splenectomy for immune thrombocytopenia: Down but not out. Blood. 2018 Mar 15;131(11):1172-82.

Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA, American Society of Hematology. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011 Apr 21;117(16):4190-207.

Vianelli N, Galli M, De Vivo A, Intermesoli T, Giannini B, Mazzucconi MG, et al. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: Long-term results of 402 cases. Haematologica. 2005;90(1):72–7.

Mcmillan R, Durette C. Long-term outcomes in adults with chronic ITP after splenectomy failure. Blood. 2004 Aug 15;104(4):956-60.

Vianelli N, Palandri F, Polverelli N, Stasi R, Joelsson J, Johansson E, et al. Splenectomy as a curative treatment for immune thrombocytopenia: a retrospective analysis of 233 patients with a minimum follow up of 10 years. Haematologica. 2013 Jun;98(6):875-80.

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 Sep;95(9):1429-34.

Saleh MN, Bussel JB, Cheng G, Meyer O, Bailey CK, Arning M. Safety and efficacy of eltrombopag for treatment of chronic immune thrombocytopenia: results of the long-term, open-label EXTEND study. Blood. 2013 Jan 17;121(3):537-45.

Kuter DJ, Bussel JB, Newland A, Baker RI, Lyons M, Wasser J, et al. Long-term treatment with romiplostim in patients with chronic immune thrombocytopenia: safety and efficacy. Br J Haematol. 2013 May;161(3):411-23.

Published
2020-04-30
How to Cite
Kara-Jovanović, A., & Suvajdžić-Vuković, N. (2020). Clinical Presentation and Therapy of Primary Immune Thrombocytopenia Resistant to Splenectomy. International Journal of Medical Students, 8(1), 11-14. https://doi.org/10.5195/ijms.2020.436
Section
Original Article