Abstract
Introduction
We investigated short and long-term remission rates after splenectomy in patients with primary and secondary autoimmune hemolytic anemia (AIHA).
Methods
All adults who underwent splenectomy for primary or secondary AIHA at a single center (2004–2018) were retrospectively reviewed. Short-term response was determined at 30-day postoperatively and long-term at one year. Complete response was defined as hemoglobin > 10 g/dL without hemolysis, transfusions, or need for additional medical therapy for > 6 months.
Results
Short-term complete response was attained in 22 of 36 patients (61%), partial response in 3 (8%), no response in 11 (31%). The response rate at 1 year was complete in 14/36 (39%), partial in 14 (39%), and 8 non-response (22%). At last available follow-up (median 33.1 months (IQR 19–59), 16/37 patients had experienced a complete response (43%), 14 partial response (38%), 7 non-response (19%). 80% of partial responders with primary AIHA required maintenance therapy compared to 100% with secondary AIHA.
Conclusion
Splenectomy is associated with short- and long-term improvement in anemia and hemolysis in the majority of patients with AIHA. Immunosuppressants remain important supplemental therapy.
Similar content being viewed by others
References
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C (2014) Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients. Blood. https://doi.org/10.1182/blood-2014-06-583021
Bradley C, Gehrs RCF (2018) Autoimmune hemolytic anemia. Am J Hematol. https://doi.org/10.1016/j.mcna.2016.09.007
Giannouli S, Voulgarelis M, Ziakas PD, Tzioufas AG (2006) Anaemia in systemic lupus erythematosus: from pathophysiology to clinical assessment. Ann Rheum Dis. https://doi.org/10.1136/ard.2005.041673
Böttiger LE, Westerholm B (1973) Acquired haemolytic anaemia: I. Incidence and aetiology. Acta Med Scand. https://doi.org/10.1111/j.0954-6820.1973.tb10567.x
Liebman HA, Weitz IC (2017) Autoimmune hemolytic anemia. Med Clin NA. https://doi.org/10.1016/j.mcna.2016.09.007
Dierickx D, Kentos A, Delannoy A (2015) The role of rituximab in adults with warm antibody autoimmune hemolytic anemia. Blood. https://doi.org/10.1182/blood-2015-01-588392
Barcellini W, Zaja F, Zaninoni A et al (2013) Sustained response to low-dose rituximab in idiopathic autoimmune hemolytic anemia. Eur J Haematol. https://doi.org/10.1111/ejh.12199
Sys J, Provan D, Schauwvlieghe A, Vanderschueren S, Dierickx D (2017) The role of splenectomy in autoimmune hematological disorders: outdated or still worth considering? Blood Rev. https://doi.org/10.1016/j.blre.2017.01.001
Coon WW (1985) Splenectomy in the treatment of hemolytic anemia. Arch Surg. https://doi.org/10.1001/archsurg.1985.01390290099017
Allgood JW, Chaplin H (1967) Idiopathic acquired autoimmune hemolytic anemia: a review of forty-seven cases treated from 1955 through 1965. Am J Med. https://doi.org/10.1016/0002-9343(67)90168-4
Akpek G, McAneny D, Weintraub L (1999) Comparative response to splenectomy in coombs-positive autoimmune hemolytic anemia with or without associated disease. Am J Hematol. https://doi.org/10.1002/(sici)1096-8652(199906)61:2%3c98::aid-ajh4%3e3.0.co;2-g
Zanella A, Barcellini W (2014) Treatment of autoimmune hemolytic anemias. Haematologica. https://doi.org/10.3324/haematol.2014.114561
Dierickx D, Verhoef G, Van Hoof A, Mineur P, Roest A, Triffet A et al (2009) Rituximab in autoimmune haemolytic anaemia and immune thrombocytopenic purpura: a Belgian retrospective multicentric study. J Intern Med. https://doi.org/10.1111/j.1365-2796.2009.02126.x
Crowther M, Chan YLT, Garbett IK, Lim W, Vickers MA, Crowther MA (2011) Evidence-based focused review of the treatment of idiopathic warm immune hemolytic anemia in adults. Blood. https://doi.org/10.1182/blood-2011-05-347708
Dindo D, Demartines N, Clavien PA (2004) Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg. https://doi.org/10.1097/01.sla.0000133083.54934.ae
Rashid SA (2014) Accessory spleen: prevalence and multidetector CT appearance. Malays J Med Sci 21(4):18–23
Bowdler AJ (1976) The role of the spleen and splenectomy in autoimmune hemolytic disease. Semi Hematol 13(4):335–348
Zupanska B, Sylwestrowicz T, Pawelski S (1981) The results of prolonged treatment of autoimmune haemolytic anaemia. Haematologia (Budap) 14(4):425–433
Rodeghiero F, Ruggeri M (2008) Is splenectomy still the gold standard for the treatment of chronic ITP? Am J Hematol. https://doi.org/10.1002/ajh.21109,February2008
Palandri F, Polverelli N, Sollazzo D et al (2016) Have splenectomy rate and main outcomesof ITP changed after the introduction of new treatments? A monocentric study inthe outpatient setting during 35 years. Am J Hematol. https://doi.org/10.1002/ajh.24310
Reynaud Q, Durieu I, Dutertre M et al (2015) Efficacy and safety of rituximab in auto-immune hemolytic anemia: a meta-analysis of 21 studies. Autoimmun Rev. https://doi.org/10.1016/j.autrev.2014.11.014
Gómez-Almaguer D, Solano-Genesta M, Tarín-Arzaga L et al (2010) Low-dose rituximab and alemtuzumab combination therapy for patients with steroid-refractory autoimmune cytopenias. Blood. https://doi.org/10.1182/blood-2010-06-291831
Rossi G, Gramegna D, Paoloni F et al (2018) Short course of bortezomib in anemic patients with relapsed cold agglutinin disease: a phase 2 prospective GIMEMA study. Blood. https://doi.org/10.1182/blood-2018-03-835413
Jasinski S, Weinblatt ME, Glasser CL (2017) Sirolimus as an effective agent in the treatment of immune thrombocytopenia (ITP) and evans syndrome (ES): a single institution’s experience. J Pediatr Hematol Oncol. https://doi.org/10.1097/MPH.0000000000000818
Park JA, Lee HH, Kwon HS, Baik CR, Song SA, Lee JN (2016) Sirolimus for refractory autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation: a case report and literature review of the treatment of post-transplant autoimmune hemolytic anemia. Transfus Med Rev. https://doi.org/10.1016/j.tmrv.2015.09.001
Neave L, Wilson A, Lissack M, Scully M (2018) Severe refractory idiopathic warm autoimmune haemolytic anaemia responsive to complement inhibition with eculizumab. BMJ Case Rep. https://doi.org/10.1136/bcr-2018-226429
Ma K, Caplan S (2016) Refractory IgG warm autoimmune hemolytic anemia treated with eculizumab: a novel application of anticomplement therapy. Case Rep Hematol. https://doi.org/10.1155/2016/9181698
Schuetz C, Hoenig M, Moshous D, Weinstock C, Castelle M, Bendavid M, Shimano K, Tolbert V, Schulz AS, Dvorak CC (2018) Daratumumab in life-threatening autoimmune hemolytic anemia following hematopoietic stem cell transplantation. Blood Adv. https://doi.org/10.1182/bloodadvances.2018020883
Hess J, Su L, Nizzi F, Beebe K, Magee K, Salzberg D, Stahlecker J, Miller HK, Adams RH, Ngwube A (2018) Successful treatment of severe refractory autoimmune hemolytic anemia after hematopoietic stem cell transplant with abatacept. Transfusion. https://doi.org/10.1111/trf.14907
Allan-Schrimscher C, Beebe KL, Magee KH, McNulty A, Salzberg D, Stahlecker J, Miller HK, Adams RH, Ngwube A (2018) Successful treatment of severe autoimmune hemolytic anemia after hematopoietic stem cell transplant with abatacept: a case series. Tansplant Cell Ther. https://doi.org/10.1016/j.bbmt.2017.12.059
Balague C, Targarona EM, Cerdan G, Novell J, Montero O, Bendahan G, Garcia A, Pey A, Vela S, Diaz M, Trias M (2004) Long-term outcome after laparoscopic splenectomy related to hematologic diagnosis. Surgl Endosc. https://doi.org/10.1007/s00464-003-9092-y
Bisharat N, Omari H, Lavi I, Raz R (2001) Risk of infection and death among post-splenectomy patients. J Infect. https://doi.org/10.1053/jinf.2001.0904
Krauth MT, Lechner K, Neugebauer EAM, Pabinger I (2008) The postoperative splenic/portal vein thrombosis after splenectomy and its prevention: AN unresolved issue. Haematologica. https://doi.org/10.3324/haematol.12682,June12
Kyaw MH, Holmes EM, Toolis F et al (2006) Evaluation of severe infection and survival after splenectomy. Am J Med. https://doi.org/10.1016/j.amjmed.2005.07.044,March
Author information
Authors and Affiliations
Contributions
(I) Conception and design: RAM, SM, SR; (II) Administrative support: None; (III) Provision of study materials or patients: SR, MR; (IV) Collection and assembly of data: SM; RAM, SZ, AF; (V) Data analysis and interpretation: SM, AF, RN, SZ, CP, MR, SR; (VI) Manuscript writing: SM, AF, AI, DK, AP, CP, MR, SR; (VII) Final approval of manuscript: All authors.
Corresponding author
Ethics declarations
Disclosures
Ajita Prabhu MD reports personal fees from Verb Surgical, personal fees from CMR Surgical, grants and personal fees from Intuitive Surgical, outside the submitted work. Michael Rosen MD reports personal fees from ACHQC, outside the submitted work. Aldo Fafaj MD, reports grants from AHSQC, outside the submitted work. Sara Maskal MD, Raha Al Marzooqi MD, Samuel Zolin MD, Robert Naples DO, Advait Iyer DO, Clayton Petro MD, David Krpata MD, Steven Rosenblatt MD have no conflicts of interest or financial ties to disclose.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Maskal, S., Al Marzooqi, R., Fafaj, A. et al. Clinical and surgical outcomes of splenectomy for autoimmune hemolytic anemia. Surg Endosc 36, 5863–5872 (2022). https://doi.org/10.1007/s00464-022-09116-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00464-022-09116-x