Session Type: Abstract Submissions (ACR)
Acute presentation of severe autoinmune thrombocytopenia, and hemolytic anemia in systemic lupus erythematosus (SLE) is associated with high mortality. Splenectomy is the second line of treatment.
The aim of this study is to investigate the efficacy and safety of splenectomy in refractory thrombocytopenia and hemolytic anemia associated or not with SLE.
From January 2004 to April 2014, 34 patients underwent splenectomy due to severe autoimmune thrombocytopenia and/or hemolytic anemia. The mean age of patients were 34.6 years old (range 18-62 y.o.) and twenty eight patients were female (82.35%). The patients were divided into two groups: Group 1: 18 patients with thrombocytopenia associated with SLE (9), SLE plus antiphospholipid syndrome, APS, (6), and primary APS (3). Group 2: 16 patients without SLE: Fisher-Evans Syndrome (2), and hemolytic anemia (14). All patients had refractory hematological manifestations, which 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 hemoglobin ≤ 6 grams per ml) despite to conventional treatment in a 6 months period. The response to splenectomy 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 ml. The inmediate response were evaluated after 7 days. The mean of follow up were 28.5 months (range3-96 months). Statistical Analysis: descriptive stadistics and Chi square Test.
Open splenectomy was performed in 15/34 patients (44.11%) and laparoscopy in 19/34 patients, 3 converted to open surgery. The complete response were observed in 15/34, (44.11%).Group 1, 4/18 (22.2%) and Group 2, 11/16, (68.8%) (p= .006). After 30 days of surgery a complete response were observed in 11/18, (61.1%) for Group 1 and 13/16, (81.2%) Group 2 (p=NS). The complications in the inmediate post-operative period were observed in 6/34, 5 of Group 2 vs 1 in Group 1 (p=0.05). Infections in 3/34, one patient had bleeding and 2 had mesenteric and portal vein thrombosis respectively. In Group 1, the mean of period between hematologic manifestations and surgery was 17.8 months and in Group 2, 16.5 months. After follow up, in Group 1, the relapse were observed in 7/18 patients, and in Group 2, 3/16. In Group 1, steroids were reduced in 13/18 patients, and 14/16 patients of the Group 2.
This study suggest that patients with SLE, SLE plus APS and primary APS have a similar response to splenectomy compared with organ-specific autoinmune diseases despite a partial response in the inmediate period. Patients with organ-specific autoinmune diseases had a significant increase of complications. The splenectomy is safety and effective in severe and refractory hematologic manifestations.The surgery approach has not influence in the prognosis of splenectomy.
L. J. Jara,
M. A. Saavedra,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/splenectomy-in-systemic-lupus-erythematosus-and-autoimmune-hematological-diseases-a-comparative-analysis/