Abstract
Since the role of splenectomy in treating thrombocytopenia associated with systemic lupus erythematosus has been controversial, the study was focused on determining the efficacy of splenectomy in the SLE-associated thrombocytopenia. Between 1980 and 2008, 11 patients with SLE underwent splenectomy for treating their thrombocytopenia. Surgical indications, operative mortality and morbidity, and haematological outcomes were followed in both short term (first 30 days) and long term (last recorded platelet count, last contact, or death). Indications for splenectomy included: thrombocytopenia refractory to (63.7%), dependent on (27.3%), or patient intolerance of (9%) medical treatments. Perioperative mortality and morbidity was 0%. The overall rate of early partial or complete response rate to splenectomy was 100%. After a median follow-up of 36 months, 9 (81.9%) patients had sustained complete or partial response without relapse. Eight (72.8%) of these patients required adjunctive medical therapy, whereas the other 1 (9%) did not. The remaining 2 (18.2%) patients relapsed, could not been subsequently salvaged to at least partial response with further treatments. The overall PR or CR to splenectomy combined with medical therapy was 81.8%. Splenectomy should be considered as a safe and efficacious therapy for the severe thrombocytopenia associated with SLE in some selected patients.
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References
Rabinowitz Y, Dameshek W (1960) Systemic lupus erythematosus after “idiopathic” thrombocytopenic purpura: a review. Ann Intern Med 52:1–28
Reveille JD, Bartolucci A, Alarcon GS (1990) Prognosis in systemic lupus erythematosus. Negative impact of increasing age at onset, black race, and thrombocytopenia, as well as causes of death. Arthritis Rheum 33(1):37–48
Ward MM, Pyun E, Studenski S (1996) Mortality risks associated with specific clinical manifestations of systemic lupus erythematosus. Arch Intern Med 156(12):1337–1344
Pujol M, Ribera A, Vilardell M, Ordi J, Feliu E (1995) High prevalence of platelet autoantibodies in patients with systemic lupus erythematosus. Br J Haematol 89(1):137–141
Dixon RH, Rosse WF (1975) Platelet antibody in autoimmune thrombocytopenia. Br J Haematol 31(2):129–134
Gruenberg JC, VanSlyck EJ, Abraham JP (1986) Splenectomy in systemic lupus erythematosis. Am Surg 52(7):366–370
Budman DR, Steinberg AD (1977) Hematologic aspects of systemic lupus erythematosus. Current concepts. Ann Intern Med 86(2):220–229
Lurie DP, Kahaleh MB (1982) Pulse corticosteroid therapy for refractory thrombocytopenia in systemic lupus erythematosus. J Rheumatol 9(2):311–314
Homan WP, Dineen P (1978) The role of splenectomy in the treatment of thrombocytopenic purpura due to systemic lupus erythematosus. Ann Surg 187(1):52–56
Cooper MR, Hansen KS, Maynard CD, Elrod IW, Spurr CL (1972) Platelet survival and sequestration patterns in thrombocytopenic disorders. Radiology 102(1):89–100
West SG, Johnson SC (1988) Danazol for the treatment of refractory autoimmune thrombocytopenia in systemic lupus erythematosus. Ann Intern Med 108(5):703–706
Gelfand J, Truong L, Stern L, Pirani CL, Appel GB (1985) Thrombotic thrombocytopenic purpura syndrome in systemic lupus erythematosus: treatment with plasma infusion. Am J Kidney Dis 6(3):154–160
Vos JJ, van Aken WG, Engelfriet CP, von Dem Borne AE (1985) Intravenous gammaglobulin therapy in idiopathic thrombocytopenic purpura. Results with the Netherlands Red Cross immunoglobulin preparation. Vox Sang 49(2):92–100
Ahn YS, Harrington WJ, Seelman RC, Eytel CS (1974) Vincristine therapy of idiopathic and secondary thrombocytopenias. N Engl J Med 291(8):376–380
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
Baldini MG (1972) Idiopathic thrombocytopenic purpura and the ITP syndrome. Med Clin North Am 56(1):47–64
Dameshek HL, Ellis LD (1975) Hematologic indications for splenectomy. Surg Clin North Am 55(2):253–275
Gadenstatter M, Lamprecht B, Klingler A, Wetscher GJ, Greil R, Schmid T (2002) Splenectomy versus medical treatment for idiopathic thrombocytopenic purpura. Am J Surg 184(6):606–609 (discussion pp 609–610)
Katkhouda N, Hurwitz MB, Rivera RT, Chandra M, Waldrep DJ, Gugenheim J et al (1998) Laparoscopic splenectomy: outcome and efficacy in 103 consecutive patients. Ann Surg 228(4):568–578
Jacobs P, Wood L, Dent DM (1986) Splenectomy and the thrombocytopenia of systemic lupus erythematosus. Ann Intern Med 105(6):971–972
Raguin G, Le Thi Huong D, Piette JC, Bletry O, Renou P, Guillevin L et al (1989) Splenectomy can cure thrombocytopenia of systemic lupus erythematosus. Presse Med 18(35):1739–1742
Hall S, McCormick JL Jr, Greipp PR, Michet CJ Jr, McKenna CH (1985) Splenectomy does not cure the thrombocytopenia of systemic lupus erythematosus. Ann Intern Med 102(3):325–328
Rivero SJ, Alger M, Alarcon-Segovia D (1979) Splenectomy for hemocytopenia in systemic lupus erythematosus. A controlled appraisal. Arch Intern Med 139(7):773–776
Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725
Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH (1992) Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum 35(6):630–640
Goldbach-Mansky R, Wilson M, Fleischmann R, Olsen N, Silverfield J, Kempf P et al. (2009) Comparison of Tripterygium wilfordii Hook F versus sulfasalazine in the treatment of rheumatoid arthritis: a randomized trial. Ann Intern Med 151(4):229–40, W49–W51
Yee CS, Isenberg DA, Prabu A, Sokoll K, Teh LS, Rahman A et al (2008) BILAG-2004 index captures systemic lupus erythematosus disease activity better than SLEDAI-2000. Ann Rheum Dis 67(6):873–876
Uribe AG, Vila LM, McGwin G Jr, Sanchez ML, Reveille JD, Alarcon GS (2004) The systemic lupus activity measure-revised, the Mexican Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and a modified SLEDAI-2 K are adequate instruments to measure disease activity in systemic lupus erythematosus. J Rheumatol 31(10):1934–1940
Karpatkin S, Siskind GW (1969) In vitro detection of platelet antibody in patients with idiopathic thrombocytopenic purpura and systemic lupus erythematosus. Blood 33(6):795–812
Alarcon-Segovia D (2002) Splenectomy has a limited role in the management of lupus with thrombocytopenia. J Rheumatol 29(1):1–2
Mestanza-Peralta M, Ariza-Ariza R, Cardiel MH, Alcocer-Varela J (1997) Thrombocytopenic purpura as initial manifestation of systemic lupus erythematosus. J Rheumatol 24(5):867–870
Wagner DS, Schlueter TM (1984) Splenectomy: indications, complications and mortality. Ohio State Med J 80(8):610–613
Hakim AJ, Machin SJ, Isenberg DA (1998) Autoimmune thrombocytopenia in primary antiphospholipid syndrome and systemic lupus erythematosus: the response to splenectomy. Semin Arthritis Rheum 28(1):20–25
Arnal C, Piette JC, Leone J, Taillan B, Hachulla E, Roudot-Thoraval F et al (2002) Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases. J Rheumatol 29(1):75–83
Visudhiphan S, Ketsa-Ard K, Piankijagum A, Tumliang S (1985) Blood coagulation and platelet profiles in persistent post-splenectomy thrombocytosis. The relationship to thromboembolism. Biomed Pharmacother 39(6):264–271
Lehembre S, Macario-Barrel A, Musette P, Carvalho P, Joly P (2006) Rituximab treatment for immune thrombocytopenia associated with systemic lupus erythematosus. Ann Dermatol Venereol 133(1):53–55
Acknowledgments
This study was partially supported by grant from NSFC (Natural Science Foundation of China) 30830094/C080701 to Dr. Guixiu Shi.
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Li, R., Liu, G., Wang, K. et al. Splenectomy for thrombocytopenia associated with systemic lupus erythematosus in 11 Chinese patients. Rheumatol Int 31, 9–15 (2011). https://doi.org/10.1007/s00296-009-1207-x
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DOI: https://doi.org/10.1007/s00296-009-1207-x