ORIGINAL ARTICLE Splenectomy in systemic lupus erythematosus and autoimmune hematologic disease: a comparative analysis 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 Received: 6 September 2017 /Revised: 29 December 2017 /Accepted: 3 January 2018 # International League of Associations for Rheumatology (ILAR) 2018 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 Introduction Systemic lupus erythematosus (SLE) is an autoimmune dis- ease, with multisystemic involvement, ranging from mild to life threatening, being the exacerbations and remissions the most frequent pattern of evolution [1, 2]. Between 8 and 20% of SLE patients may be affected by autoimmune throm- bocytopenia (AT) and autoimmune hemolytic anemia (AIHA) occurred in 5–10% [3–5]. On the other hand, Evans syndrome (AIHA and AT) has been found in 0.47% of SLE patients [6]. In patients without SLE, AIHA has an incidence in adults of 0.8–3 per 10 5 /year, a prevalence of 17:100,000 and mortality rate of 11% [7]. In SLE patients, AT and AIHA can appear without clinical consequences or as an acute and serious illness with high mortality [1, 8]. In Latin American patients with SLE, AIHA was found in 5.5% and AT in 16.3%. Of interest, AIHA contributed independently to damage accrual and diminished survival [9]. In addi- tion, AT and AIHA are common features in patients with antiphospholipid syndrome (APS), although severe AT and AIHA are rare in APS [10, 11]. * Luis J. Jara luis_jara_quezada@hotmail.com 1 Surgery Department, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City, Mexico 2 Education Division, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City, Mexico 3 Clinical Research Unit, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City, Mexico 4 Research Division, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City, Mexico 5 General Direction, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City, Mexico 6 Direction of Education and Research, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City, Mexico Clinical Rheumatology https://doi.org/10.1007/s10067-018-3979-4