The Study Of Splenectomy For Thrombocytopenic And Leucocytopenia Associated With Rheumatic Disease

Objective: To determine the efficacy of splenectomy for treating thrombocytopenia and leucocytopenia associated with rheumatic disease.Background: Splenectomy is efficient in some hematonosis such as hereditary spherocytosis, idiopathic thrombocytopenic purpura, etc. Spencer Wells successfully removed a spleen for hereditary spherocytosis in 1887, and Kaznelson et al in 1916 performed the first splenectomy for thrombocytopenic purpura. From then, more and more researches were done to study the function of spleen. Growing knowledge of the function of spleen has allowed application of splenectomy to other hematologic disorders both primary and secondary to other diseases. But we have few materials on the role of splenectomy in the treatment of rheumatic disease. It has been controversial in the rheumatisant population.Method: Between 1987 and 2007, 9 patients with systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS) underwent splenectomy for thrombocytopenia and leucocytopenia. The diagnosis of SLE was based on current criteria set by the American College of Rheumatology in1997, and SS on the criteria set in 2002. Surgical indications,operative mortality, symptoms and hematologic outcomes were followed in both the short-term (first 30 days) and the long-term (last recorded platelet count, last contact). Efficacy of splenectomy for thrombocytopenia was rated as: excellent response (platelets≥100×10~9/L, no hemorrhage for at least 3 months), favourable response (platelets 50-149×10~9/L or increase＞30×10~9 /L, no or almost no hemorrhage for at least 2 months), improvement (blood platelets count increases, symptom of hemorrhage improves for 2 weeks at least), or no response (blood platelets count and symptoms don't improve at all times). Efficacy of splenectomy for leukocytopenia was rated as: excellent response (WBC＞4.0×10~9/L or increases by one time, symptoms improve excellently), effective (increasing of WBC＞0.5×10~9/L and symptoms improve), no response (increasing of WBC＜0.5×10~9/L, symptoms don't improve or get worse).Results: Indications for splenectomy included: hematocytopenia refractory to medical treatments (5/9), dependent on glucocorticosteroid (3/9), and patient intolerance of medical treatments (1/9). Perioperative mortality was 0%, and after a median of 1.7 years, no patient died; Only one patient infectioned just after the operation and cured through anti-infection treatment. Early response rate to splenectomy was 89%. After a median follow-up of 1.7 years, six (67%) patients had sustained complete or partial response without relapse, and they were all SLE patients. Five (83%) of these patients required adjunctive medical therapy, whereas the other one did not. The remaining one SS relapsed while diagnosed subacute cutaneous lupus erythematosus(SCLE) after complete response for five months, but salvaged with further medical treatements. The overall response rate to splenectomy combined with medical therapy was 89%.Conclusion: Splenectomy should be considered safe and efficacious for thrombocytopenia and leucocytopenia associated with rheumatic disease, and can be a treatment choose when disease refractory to, dependent on, or patient intolerance of medical treatments.