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Clinical Rheumatology

, Volume 37, Issue 4, pp 943–948 | Cite as

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

  • Nahim Barron
  • Jesús Arenas-Osuna
  • Gabriela Medina
  • María Pilar Cruz-Dominguez
  • Fernando González-Romero
  • José Arturo Velásques-García
  • Ernesto Alonso Ayala-López
  • Luis J. Jara
Original Article

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.

Keywords

Antiphospholipid syndrome Hemolytic anemia Splenectomy Systemic lupus erythematosus Thrombocytopenia 

Notes

Acknowledgments

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

Compliance with ethical standards

Disclosures

None.

References

  1. 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 CrossRefPubMedGoogle Scholar
  2. 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 CrossRefPubMedPubMedCentralGoogle Scholar
  3. 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 CrossRefPubMedGoogle Scholar
  4. 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 CrossRefPubMedGoogle Scholar
  5. 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 CrossRefPubMedGoogle Scholar
  6. 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 CrossRefPubMedPubMedCentralGoogle Scholar
  7. 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 CrossRefGoogle Scholar
  8. 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 CrossRefPubMedGoogle Scholar
  9. 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 CrossRefPubMedGoogle Scholar
  10. 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 CrossRefGoogle Scholar
  11. 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 CrossRefPubMedGoogle Scholar
  12. 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 CrossRefPubMedPubMedCentralGoogle Scholar
  13. 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):75–83PubMedGoogle Scholar
  14. 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 CrossRefPubMedGoogle Scholar
  15. 15.
    Zanella A, Barcellini W (2014) Treatment of autoimmune hemolytic anemias. Haematologica 99(10):1547–1554.  https://doi.org/10.3324/haematol.2014.114561 CrossRefPubMedPubMedCentralGoogle Scholar
  16. 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 CrossRefPubMedGoogle Scholar
  17. 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 CrossRefPubMedGoogle Scholar
  18. 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. 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 CrossRefPubMedGoogle Scholar
  20. 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):1867–1871PubMedGoogle Scholar
  21. 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 CrossRefPubMedGoogle Scholar
  22. 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 CrossRefPubMedGoogle Scholar
  23. 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 CrossRefPubMedGoogle Scholar
  24. 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 CrossRefGoogle Scholar
  25. 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 CrossRefPubMedGoogle Scholar
  26. 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 CrossRefPubMedGoogle Scholar
  27. 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 CrossRefPubMedPubMedCentralGoogle Scholar
  28. 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 CrossRefPubMedPubMedCentralGoogle Scholar
  29. 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 CrossRefPubMedGoogle Scholar
  30. 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 CrossRefGoogle Scholar
  31. 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 CrossRefPubMedGoogle Scholar
  32. 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 CrossRefGoogle Scholar
  33. 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 CrossRefPubMedGoogle Scholar
  34. 34.
    Jankulovski N, Antovic S, Kuzmanovska B, Mitevski A (2014) Splenectomy for haematological disorders. Pril (Makedon Akad Nauk Umet Odd Med Nauki) 35:181–187 616.15:611.411-089.873Google Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2018

Authors and Affiliations

  • Nahim Barron
    • 1
  • Jesús Arenas-Osuna
    • 2
  • Gabriela Medina
    • 3
  • María Pilar Cruz-Dominguez
    • 4
  • Fernando González-Romero
    • 1
  • José Arturo Velásques-García
    • 1
  • Ernesto Alonso Ayala-López
    • 5
  • Luis J. Jara
    • 6
  1. 1.Surgery Department, Hospital de Especialidades Centro Médico La RazaInstituto Mexicano del Seguro Social (IMSS)Mexico CityMexico
  2. 2.Education Division, Hospital de Especialidades Centro Médico La RazaInstituto Mexicano del Seguro Social (IMSS)Mexico CityMexico
  3. 3.Clinical Research Unit, Hospital de Especialidades Centro Médico La RazaInstituto Mexicano del Seguro Social (IMSS)Mexico CityMexico
  4. 4.Research Division, Hospital de Especialidades Centro Médico La RazaInstituto Mexicano del Seguro Social (IMSS)Mexico CityMexico
  5. 5.General Direction, Hospital de Especialidades Centro Médico La RazaInstituto Mexicano del Seguro Social (IMSS)Mexico CityMexico
  6. 6.Direction of Education and Research, Hospital de Especialidades Centro Médico La RazaInstituto Mexicano del Seguro Social (IMSS)Mexico CityMexico

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