Skip to main content
Log in

Splenectomy in systemic lupus erythematosus and autoimmune hematologic disease: a comparative analysis

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

The objective of the study is to analyze the efficacy and safety of splenectomy in the management of refractory autoimmune thrombocytopenia (AT)/autoimmune hemolytic anemia (AIHA) associated or not with systemic lupus erythematosus. Thirty-four patients after splenectomy due to severe AT and/or AIHA were divided into group 1 (G1) 18 SLE/APS patients: 9 AT/SLE patients, 6 SLE/antiphospholipid syndrome (APS), and 3 primary APS. Group 2 (G2): 16 patients without SLE/APS: 2 Fisher-Evans syndrome and 14 AIHA. Surgery approach when (1) platelets ≤ 50,000/ml despite 2 weeks on medical therapy, (2) medically dependent, and (3) medically intolerant or after two hemolytic crises in AIHA patients. Splenectomy response: (1) complete (CR): ≥ 150,000 platelets/ml, (2) partial: 50,000–149,000/ml, or (3) none: ≤ 50,000/ml. CR for AIHA: hemoglobin ≥9 g/dl. Statistical analysis: descriptive statistics and chi-square test. The mean age was 34.6 years; mean follow-up: 28.5 months. Open splenectomy in 15/34 vs laparoscopy in 19/34 (p = NS). CR in 15/34, G1: 4/18, G2: 11/16, (p = 0.006). Complications in 6/34, 5 from G2 vs 1 from G1 (p = 0.05). Relapse in 7/18 patients in G1 and 3/16 in G2 (p = 0.05). Open and laparoscopic splenectomies in SLE and AT patients are as effective as in those without SLE; however, patients with SLE and APS had more relapses.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Ward MM, Pyus E, Studenski S (1996) Mortality risks associated with specific clinical manifestations of systemic lupus erythematosus. Arch Intern Med 156(12):1337–1344. https://doi.org/10.1001/archinte.1996.00440110109015

    Article  CAS  PubMed  Google Scholar 

  2. Györi N, Giannakou I, Chatzidionysiou K, Magder L, van Vollenhoven RF, Petri M (2017) Disease activity patterns over time in patients with SLE: analysis of the Hopkins Lupus Cohort. Lupus Sci Med 4(1):e000192. https://doi.org/10.1136/lupus-2016-000192

    Article  PubMed  PubMed Central  Google Scholar 

  3. Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Mejía JC, Aydintug AO, Chwalinska-Sadowska H, de Ramón E, Fernández-Nebro A, Galeazzi M, Valen M, Mathieu A, Houssiau F, Caro N, Alba P, Ramos-Casals M, Ingelmo M, Hughes GR, European Working Party on Systemic Lupus Erythematosus (2003) Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine 82(5):299–308. https://doi.org/10.1097/01.md.0000091181.93122.55

    Article  PubMed  Google Scholar 

  4. Alarcon GS, McGwin G Jr, Petri M, Reveille JD, Ramsey-Goldman R, Kimberly RP, PROFILE Study Group (2002) Baseline characteristics of a multiethnic lupus cohort: PROFILE. Lupus 11(2):95–101. https://doi.org/10.1191/0961203302lu155oa

    Article  CAS  PubMed  Google Scholar 

  5. Wang F, Wang CL, Tan CT, Manivasagar M (1997) Systemic lupus erythematosus in Malaysia: a study of 539 patients and comparison of prevalence and disease expression in different racial and gender groups. Lupus 6(3):248–253. https://doi.org/10.1177/096120339700600306

    Article  CAS  PubMed  Google Scholar 

  6. Zhang L, Wu X, Wang L, Li J, Chen H, Zhao Y, Zheng W (2016) Clinical features of systemic lupus erythematosus patients complicated with Evans syndrome: a case-control, single center study. Medicine 95(15):e3279. https://doi.org/10.1097/MD.0000000000003279

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Lechner K, Jager U (2010) How I treat autoimmune hemolytic anemias in adults. Blood 16(11):1831–1838. https://doi.org/10.1182/blood-2010-03-259325

    Article  Google Scholar 

  8. Patel N, Mody GM (2014) Acute presentation of thrombocytopaenia in systemic lupus erythematosus is associated with a high mortality in South Africa. Lupus 23(2):204–212. https://doi.org/10.1177/0961203313512009

    Article  CAS  PubMed  Google Scholar 

  9. González-Naranjo LA, Betancour OM, Alarcón GS, Ugarte-Gil MF, Jaramillo-Arroyave D, Wojdyla D et al (2016) Features associated with hematologic abnormalities and their impact in patients with systemic lupus erythematosus: data from a multiethnic Latin American cohort. Semin Arthritis Rheum 45:675–683. https://doi.org/10.1016/j.semarthrit.2015.11.003

    Article  PubMed  Google Scholar 

  10. Delgado Alves J, Inanc M, Diz-Kucukkaya R, Grima B, Soromenho F, Isenberg DA (2004) Thrombotic risk in patients submitted to splenectomy for systemic lupus erythematosus and antiphospholipid antibody syndrome-related thrombocytopenia. Eur J Inter Med 15(3):162–167. https://doi.org/10.1016/j.ejim.2004.03.005

    Article  Google Scholar 

  11. Kolasinski SL (2006) Prevalence of secondary hematologic disorders in the antiphospholipid syndrome: impact on coagulation risk. Curr Rheumatol Rep 8(2):95–99. https://doi.org/10.1007/s11926-006-0048-9

    Article  CAS  PubMed  Google Scholar 

  12. You NY, Tefferi A, Nagorney M (2004) Outcome of splenectomy for thrombocytopenia associated with systemic lupus erythematosus. Ann Surg 240(2):286–292. https://doi.org/10.1097/01.sla.0000133182.92780.9c

    Article  PubMed  PubMed Central  Google Scholar 

  13. Arnal C, Piette JC, Léone J, Taillan B, Hachulla E, Roudot-Thoraval F, Papo T, Schaeffer A, Bierling P, Godeau B (2002) Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases. J Rheumatol 29(1):75–83

    CAS  PubMed  Google Scholar 

  14. Galindo M, Khamashta MA, Hughes GR (1999) Splenectomy for refractory thrombocytopenia in the antiphospholipid syndrome. Rheumatology 38(9):848–853. https://doi.org/10.1093/rheumatology/38.9.848

    Article  CAS  PubMed  Google Scholar 

  15. Zanella A, Barcellini W (2014) Treatment of autoimmune hemolytic anemias. Haematologica 99(10):1547–1554. https://doi.org/10.3324/haematol.2014.114561

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725. https://doi.org/10.1002/1529-0131(199709)40:9<1725::AID-ART29>3.0.CO;2-Y

    Article  CAS  PubMed  Google Scholar 

  17. Miyakis S, Lockshin MD, Atsumi T et al (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4(2):295–306. https://doi.org/10.1111/j.1538-7836.2006.01753.x

    Article  CAS  PubMed  Google Scholar 

  18. Bass GF et al (2014) Diagnosis and classification of autoimmune hemolytic anemia. Autoimmun Rev. https://doi.org/10.1016/j.autrev.2013.11.010

  19. Zhou J, Wu Z, Zhou Z, Wang Z, Liu Y, Huang XY, Peng B (2013) Efficacy and safety of laparoscopic splenectomy in thrombocytopenia secondary to systemic lupus erythematosus. Clin Rheumatol 32(8):1131–1138. https://doi.org/10.1007/s10067-013-2230-6

    Article  PubMed  Google Scholar 

  20. Cervera H, Jara LJ, Pizarro S, Enkerlin HL, Fernandez M, Medina F, Fraga A, Miranda JM (1995) Danazol for systemic lupus erythematosus with refractory autoimmune thrombocytopenia or Evan’s syndrome. J Rheumatol 22(10):1867–1871

    CAS  PubMed  Google Scholar 

  21. Ahn YS, Harrington WJ, Seelman RC, Eytel CS (1974) Vincristine therapy of idiopathic and secondary thrombocytopenias. N Engl J Med 291(8):376–380. https://doi.org/10.1056/NEJM197408222910802

    Article  CAS  PubMed  Google Scholar 

  22. Boumpas DT, Barez S, Klippel JH, Balow JE (1990) Intermittent cyclophosphamide for the treatment of autoimmune thrombocytopenia in systemic lupus erythematosus. Ann Intern Med 112(9):674–677. https://doi.org/10.7326/0003-4819-112-9-674

    Article  CAS  PubMed  Google Scholar 

  23. Li R, Liu G, Wang K, Liu Y, Xie Q, Liu Y, Shi G (2011) Splenectomy for thrombocytopenia associated with systemic lupus erythematosus in 11 Chinese patients. Rheumatol Int 31(1):9–15. https://doi.org/10.1007/s00296-009-1207-x

    Article  PubMed  Google Scholar 

  24. Jaime-Pérez JC, Rodríguez-Martínez M, Gómez-de-León A, Tarín-Arzaga L, Gómez-Almaguer D (2013) Current approaches for the treatment of autoimmune hemolytic anemia. Arch Immunol Ther Exp 61(5):385–395. https://doi.org/10.1007/s00005-013-0232-3

    Article  Google Scholar 

  25. Jeffries M, Hamadeh F, Aberle T, Glenn S, Kamen DL, Kelly JA, Reichlin M, Harley JB, Sawalha AH (2008) Haemolytic anemia in a multi-ethnic cohort of lupus patients: a clinical and serological perspective. Lupus 17(8):739–743. https://doi.org/10.1177/0961203308090990

    Article  CAS  PubMed  Google Scholar 

  26. Ellison EC, Fabri PJ (1983) Complications of splenectomy. Etiology, prevention, and management. Surg Clin North Am 63(6):1313–1330. https://doi.org/10.1016/s0039-6109(16)43191-9

    Article  CAS  PubMed  Google Scholar 

  27. Ikeda M, Sekimoto M, Takiguchi S, Kubota M, Ikenaga M, Yamamoto H, Fujiwara Y, Ohue M, Yasuda T, Imamura H, Tatsuta M, Yano M, Furukawa H, Monden M (2005) High incidence of thrombosis of the portal venous system after laparoscopic splenectomy: a prospective study with contrast-enhanced CT scan. Ann Surg 241(2):208–216. https://doi.org/10.1097/01.sla.0000151794.28392.a6

    Article  PubMed  PubMed Central  Google Scholar 

  28. Zhang N, Yao Y, Xue W, Wu S (2016) Early prophylactic anticoagulation for portal vein system thrombosis after splenectomy: a systematic review and meta-analysis. Biomed Rep 5(4):483–490. https://doi.org/10.3892/br.2016.755

    Article  PubMed  PubMed Central  Google Scholar 

  29. Habermalz B, Sauerland S, Decker G, Delaitre B, Gigot JF, Leandros E, Lechner K, Rhodes M, Silecchia G, Szold A, Targarona E, Torelli P, Neugebauer E (2008) Laparoscopic splenectomy: the clinical practice guidelines of the European Association for Endoscopic Surgery (EAES). Surg Endosc 22(4):821–848. https://doi.org/10.1007/s00464-007-9735-5

    Article  CAS  PubMed  Google Scholar 

  30. Dragomir M, Petrescu DGE, Manga GE, Călin GA, Vasilescu C (2016) Patients after splenectomy: old risks and new perspectives. Chirurgia (Bucur) 111(5):393–399. https://doi.org/10.21614/chirurgia.111.5.393

    Article  Google Scholar 

  31. Mok CC (2015) Current role of rituximab in systemic lupus erythematosus. Int J Rheum Dis 18(2):154–163. https://doi.org/10.1111/1756-185X.12463

    Article  CAS  PubMed  Google Scholar 

  32. Pons I, Espinosa G, Cervera R (2014) Efficacy and safety of rituximab in the treatment of primary antiphospholipid syndrome: analysis of 24 cases from the bibliography review. Med Clin (Barc) 144(3):97–104. https://doi.org/10.1016/j.medcli.2014.01.034

    Article  Google Scholar 

  33. Misra DP, Jain VK, Negi VS (2016) Splenectomy increases the subsequent risk of systemic lupus erythematosus: a word of caution. Rheumatol Int 36:277. https://doi.org/10.1007/s00296-015-3406-y

    Article  PubMed  Google Scholar 

  34. Jankulovski N, Antovic S, Kuzmanovska B, Mitevski A (2014) Splenectomy for haematological disorders. Pril (Makedon Akad Nauk Umet Odd Med Nauki) 35:181–187 616.15:611.411-089.873

    Google Scholar 

Download references

Acknowledgments

The authors thank to Dr. Bernal Humberto Esquinca Moreno for the kind supply of the spleen photograph.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Luis J. Jara.

Ethics declarations

Disclosures

None.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Barron, N., Arenas-Osuna, J., Medina, G. et al. Splenectomy in systemic lupus erythematosus and autoimmune hematologic disease: a comparative analysis. Clin Rheumatol 37, 943–948 (2018). https://doi.org/10.1007/s10067-018-3979-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-018-3979-4

Keywords

Navigation