Efficacy and safety of splenectomy in immune thrombocytopenic purpura: long-term results of 402 cases.

BACKGROUND AND OBJECTIVES Immune thrombocytopenic purpura (ITP) is an acquired autoimmune disease characterized by platelet destruction. Glucocorticoids are the first-choice treatment, resulting in a complete (CR) or partial (PR) response in 70-80% of cases. In most cases, however, response is transient or glucocorticoid-dependent. For these and for selected patients with acute refractory ITP, splenectomy may produce a good response (CR+PR) in about 60-80% of cases. We report here the long-term outcome of a large cohort of ITP splenectomized patients. DESIGN AND METHODS We retrospectively analyzed the data on 402 patients (137 males, 265 females) who underwent splenectomy for ITP between 1959 and 2002 in 22 different Hematology Centers. RESULTS Seventy-nine of the 345 (23%) responsive patients relapsed, in most cases (80%) within 48 months from splenectomy. Sixty-eight out of these 79 patients (86%) were then treated with a good response in 46/68 (68%) cases. Fifty-four of the 57 patients refractory to splenectomy and were treated, after the surgery, with a good response in 27/54 (50%) cases. Infection and thrombosis did not significantly weigh upon the outcome of the patients. Only three patients died of hemorrhage during follow-up. By multivariate analysis, the number of therapies before (p<0.01) and higher peak post-splenectomy platelet count (p<0.00001) were predictive of a favorable response to splenectomy, whereas only higher post-splenectomy peak platelet count (p<0.001) was predictive of relapse. INTERPRETATION AND CONCLUSIONS This study shows that splenectomy is a safe procedure and effective in approximately two thirds of patients with chronic ITP. Further studies are required to establish whether surgery-sparing treatments of chronic ITP, such as high-dose dexamethasone, anti-D and anti-CD20 immunoglobulins, have similar or even superior efficacy, risk and cost ratios compared to splenectomy.