Splenectomy Outcomes in Immune Cytopenias: Treatment Outcomes and Determinants of Response

Overview

Journal J Intern Med

Specialty General Medicine

Date 2023 Nov 13

PMID 37953670

Authors

Olisaemeka D Ogbue

Waled Bahaj

Tariq Kewan

Ramsha Ahmed

Danai Dima

Nakisha Willimas

Arda Durmaz

Valeria Visconte

Sara M Maskal

Carmelo Gurnari

Rosenblatt Steven

Jaroslaw P Maciejewski

Affiliations

Soon will be listed here.

Abstract

Background: Splenectomy is commonly used to treat refractory immune-mediated cytopenia, but there are no established factors that are associated with response to the procedure.

Objectives: A cohort study was conducted to evaluate the hematologic and surgical outcomes of splenectomy in adult patients with immune cytopenias and identify preoperative factors associated with response.

Methods: Data from the Cleveland Clinic Foundation for 1824 patients aged over 18 who underwent splenectomy from 2002 to 2020 were analyzed.

Results: The study found that the most common indications for splenectomy were immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia, with a median age of 55 years and median time from diagnosis to splenectomy of 11 months. Hematologic response rates were 74% overall, with relapse in 12% of cases. Postsplenectomy discordant diagnoses were present in 13% of patients, associated with higher relapse rates. Surgery-related complications occurred in 12% of cases, whereas only 3% of patients died from disease complications. On univariate analysis, preoperative factors associated with splenectomy treatment failure were ≥3 lines of pharmacologic treatment, whereas isolated thrombocytopenia, primary ITP, and age ≤40 years had a strong association with response. The multivariable regression confirmed that treatment failure with multiple lines of medical therapy was associated with the failure to respond to splenectomy.

Conclusion: Overall, the study demonstrates that splenectomy is an effective treatment option for immune-mediated cytopenias with a low complication rate.

Citing Articles

Eltrombopag for the treatment of refractory connective tissue disease-related thrombocytopenia: a pilot study of 52 cases.

Deng S, Wang B, Hu Z, Cai S, Dong L Arthritis Res Ther. 2025; 27(1):7.

PMID: 39794855 PMC: 11720346. DOI: 10.1186/s13075-024-03472-w.

Hemolytic versus malproductive anemia in large granular lymphocytic leukemia.

Ogbue O, Kewan T, Bravo-Perez C, Unlu S, Kawashima N, Williams N Leukemia. 2024; 38(8):1839-1842.

PMID: 38982262 PMC: 11286512. DOI: 10.1038/s41375-024-02323-6.

Severe, Refractory Primary Warm Autoimmune Hemolytic Anemia Requiring 90 Erythrocyte Transfusions.

Namineni N, Waldron C, Tormey C, Goshua G Ann Intern Med Clin Cases. 2024; 3(5).

PMID: 38725710 PMC: 11081177. DOI: 10.7326/aimcc.2023.1141.

Autoimmune Hemolytic Anemias: Classifications, Pathophysiology, Diagnoses and Management.

Loriamini M, Cserti-Gazdewich C, Branch D Int J Mol Sci. 2024; 25(8).

PMID: 38673882 PMC: 11049952. DOI: 10.3390/ijms25084296.

References

Kristinsson S, Gridley G, Hoover R, Check D, Landgren O . Long-term risks after splenectomy among 8,149 cancer-free American veterans: a cohort study with up to 27 years follow-up. Haematologica. 2013; 99(2):392-8. PMC: 3912973. DOI: 10.3324/haematol.2013.092460. View

Wong R, Saleh M, Khelif A, Salama A, Portella M, Burgess P . Safety and efficacy of long-term treatment of chronic/persistent ITP with eltrombopag: final results of the EXTEND study. Blood. 2017; 130(23):2527-2536. DOI: 10.1182/blood-2017-04-748707. View

Bussel J, Kaufmann C, Ware R, Woloski B . 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. DOI: 10.1002/ajh.1072. View

Riley S, ALDRETE J . Role of splenectomy in Felty's syndrome. Am J Surg. 1975; 130(1):51-2. DOI: 10.1016/0002-9610(75)90456-0. View

Hill Q, Hill A, Berentsen S . Defining autoimmune hemolytic anemia: a systematic review of the terminology used for diagnosis and treatment. Blood Adv. 2019; 3(12):1897-1906. PMC: 6595261. DOI: 10.1182/bloodadvances.2019000036. View

Park Y, Yi H, Kim C, Hong J, Park J, Lee J . Clinical Outcome and Predictive Factors in the Response to Splenectomy in Elderly Patients with Primary Immune Thrombocytopenia: A Multicenter Retrospective Study. Acta Haematol. 2016; 135(3):162-71. DOI: 10.1159/000442703. View

Sulpizio E, Raghunathan V, Shatzel J, Zilberman-Rudenko J, Worrest T, Sheppard B . Long-term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single-center retrospective study. Eur J Haematol. 2019; 104(1):55-58. PMC: 7023892. DOI: 10.1111/ejh.13336. View

Vianelli N, Palandri F, Polverelli N, Stasi R, Joelsson J, Johansson E . Splenectomy as a curative treatment for immune thrombocytopenia: a retrospective analysis of 233 patients with a minimum follow up of 10 years. Haematologica. 2012; 98(6):875-80. PMC: 3669442. DOI: 10.3324/haematol.2012.075648. View

Kojouri K, Vesely S, Terrell D, George J . 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. DOI: 10.1182/blood-2004-03-1168. View

10.

Goshua G, Sinha P, Kunst N, Pischel L, Lee A, Cuker A . Cost-effectiveness of second-line therapies in adults with chronic immune thrombocytopenia. Am J Hematol. 2022; 98(1):122-130. PMC: 9365880. DOI: 10.1002/ajh.26497. View

11.

Bagrodia N, Button A, Spanheimer P, Belding-Schmitt M, Rosenstein L, Mezhir J . Morbidity and mortality following elective splenectomy for benign and malignant hematologic conditions: analysis of the American College of Surgeons National Surgical Quality Improvement Program data. JAMA Surg. 2014; 149(10):1022-9. DOI: 10.1001/jamasurg.2014.285. View

12.

Chaturvedi S, Arnold D, McCrae K . Splenectomy for immune thrombocytopenia: down but not out. Blood. 2018; 131(11):1172-1182. PMC: 5855018. DOI: 10.1182/blood-2017-09-742353. View

13.

Subbiah V, Viny A, Rosenblatt S, Pohlman B, Lichtin A, Maciejewski J . Outcomes of splenectomy in T-cell large granular lymphocyte leukemia with splenomegaly and cytopenia. Exp Hematol. 2008; 36(9):1078-83. PMC: 3537502. DOI: 10.1016/j.exphem.2008.04.005. View

14.

Ruivard M, Caulier M, Vantelon J, Tournilhac O, Schaeffer A, Godeau B . The response to high-dose intravenous immunoglobulin or steroids is not predictive of outcome after splenectomy in adults with autoimmune thrombocytopenic purpura. Br J Haematol. 1999; 105(4):1130-2. DOI: 10.1046/j.1365-2141.1999.01464.x. View

15.

Hill Q, Stamps R, Massey E, Grainger J, Provan D, Hill A . The diagnosis and management of primary autoimmune haemolytic anaemia. Br J Haematol. 2016; 176(3):395-411. DOI: 10.1111/bjh.14478. View

16.

Hangge P, Sheaffer W, Neville M, Wasif N, Gray R, Pockaj B . The diagnostic value of splenectomy in patients without a definitive preoperative diagnosis. Am J Surg. 2018; 217(1):78-82. DOI: 10.1016/j.amjsurg.2018.05.020. View

17.

Mageau A, Terriou L, Ebbo M, Souchaud-Debouverie O, Orvain C, Graveleau J . Splenectomy for primary immune thrombocytopenia revisited in the era of thrombopoietin receptor agonists: New insights for an old treatment. Am J Hematol. 2021; 97(1):10-17. DOI: 10.1002/ajh.26378. View

18.

Tastaldi L, Krpata D, Prabhu A, Petro C, Haskins I, Perez A . Laparoscopic splenectomy for immune thrombocytopenia (ITP): long-term outcomes of a modern cohort. Surg Endosc. 2018; 33(2):475-485. DOI: 10.1007/s00464-018-6321-y. View

19.

Godeau B . Is splenectomy a good strategy for refractory immune thrombocytopenia in adults?. Br J Haematol. 2023; 203(1):86-95. DOI: 10.1111/bjh.19077. View

20.

Palandri F, Polverelli N, Sollazzo D, Romano M, Catani L, Cavo M . 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. DOI: 10.1002/ajh.24310. View