Background Acute presentation of severe autoimmune thrombocytopenia (AT) and hemolytic anemia (AHA) in systemic lupus erythematosus (SLE) is associated with high mortality. Splenectomy is the second line of treatment
Objectives Investigate the efficacy and safety of splenectomy in SLE patients compared with patients without SLE presenting acute, severe, refractory, life threatening AT and AHA.
Methods From January 2004 to April 2014, 34 patients underwent splenectomy. The patients were divided into two groups. Group 1, 18 patients with AT-SLE/APSA. Group 2: 16 patients without SLE: AT/AHA. Demographic and clinical data were analyze. Refractory hematological manifestations were defined according to Mayo Clinic Criteria as: 1. If patients did not maintain platelets ≥50,000 per ml for 2 weeks on medical therapy; 2. Medically dependent. 3. Medically intolerant. Patients with hemolytic anemia were submitted to surgery when they developed 2 hemolytic crisis (fever, jaudice, pallor, abdominal pain, and haemoglobin ≤6 gr/dL) despite to conventional treatment over a period of 6 months. After splenectomy, the response were considered for thrombocytopenia as follows: 1. Complete response: ≥150,000 platelets per ml, 2. Partial response: 50,000 to 149,000 per ml or 3. No response: <50,000 per ml. The complete response for hemolityc anemia as hemoglobin ≥9 gr per dl.The inmediate response were evaluated after 7 days. The mean of follow up were 28.5 months (range 3-96 months). Statistical Analysis: descriptive stadistics, U-Mann-Whitney test and Chi square test.
Results The mean age of 34 patients (28 female) were 34.6 years old (range 18-62 y.o.). The duration of disease:Group 1 vs Group 2: 58.3±50.6 vs 27.13±27.9 months (p<0.033). The time of AT/AHA diagnosis: Group 1 vs Group 2 (p<0.000). No difference was found in the mean of period between hematologic manifestations and surgery. Open splenectomy was performed in 15/34 patients and laparoscopy in 19/34 patients, 3 converted to open surgery (15.78%). The inmediate complete response were observed in 15/34, (67.64%). For Group1, 4/18 (22.2%) and for Group 2, 11/16, (68.8%) (p=0.006).After 30 days of surgery a complete response were observed in 11/18, (61.1%) for Group 1, and 13/16, (81.2%) for Group 2 (p=NS). The complications in the inmediate post-operative period were observed in 6/34, (17.64%), 1 from Group 1 vs 5 from Group 2 (p=0.05). Infections in 3/34 (8.82%), one patient had bleeding and 2 had mesenteric and portal vein thrombosis respectively. After follow up, in Group 1, the relapse were observed in 7/18 patients (38.9%), and in Group 2, 3/16, (18.75%) (p<0.05). The mortality was zero in both groups of patients
Conclusions This study suggest:Despite the long duration of disease, patients with SLE/APS have a similar response to splenectomy compared with AT/AHA. Patients with SLE/APS had a significant increase of relapses. The splenectomy is safety and effective in severe and refractory hematologic manifestations.
Disclosure of Interest None declared