Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications

Primary immune thrombocytopenic purpura (ITP), also referred to as idiopathic thrombocytopenic purpura, is an organ-specific autoimmune disorder in which antibodycoated or immune complex-coated platelets are destroyed prematurely by the reticuloendothelial system, resulting in peripheral blood thrombocytopenia. The disease is heterogeneous with regard to its severity and clinical course and is unpredictable in its response to therapy. Although the basic underlying pathophysiology of ITP has been known for more than 50 years, current treatment guidelines are based on expert opinion rather than on evidence because of a lack of high-quality clinical trials and research. The only patients for whom treatment is clearly required are those with severe bleeding and/or extremely low platelet counts (

Management of immune thrombocytopenic purpura in adults.

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

/pdf/efficacy-and-safety-of-splenectomy-in-immune-4zo3cqyksp.pdf

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

We performed a retrospective review of patients with immune-mediated thrombocytopenia (ITP) treated with prolonged infusions of intravenous immunoglobulin (IVIg) (1 g/kg by continuous infusion over 24 hr) and concurrent platelets (1 pheresis unit every 8 hr), to determine the response rate of this therapy. Patient inclusion criteria included clinically significant thrombocytopenia, with either active bleeding, need for anticoagulation, or a needed surgical procedure. The average pretreatment platelet count was 10,000/microl, which increased to 55,000/microl after 24 hr and 69,000/microl after 48 hr. After 24 hr, 62.7% of patients had a platelet count >50,000/microl. Bleeding was controlled initially in all patients, and those requiring a procedure experienced no bleeding complications. Over half of the patients (52.5%) required additional treatments for recurrent or refractory ITP. Six of the 21 patients requiring retreatment (29%) received IVIg and platelets again in a similar fashion, with similar results. No side effects of the combined treatment were noted. There is limited literature on the optimal dose and schedule for administration of IVIg and platelets. Our approach for administration of IVIg and platelets concurrently was associated with minimal side effects, resolution of bleeding, ability to safely undergo procedures, and rapid restoration of adequate platelet counts.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/ajh.21060

Treatment of immune-mediated thrombocytopenia purpura with concurrent intravenous immunoglobulin and platelet transfusion: a retrospective review of 40 patients.

We retrospectively investigated the association between platelet autoantibody specificity and response to intravenous immunoglobulin G (IVIG) in 17 patients with immune thrombocytopenia (ITP). Platelet-associated antibodies against glycoprotein (GP) IIb/IIIa, GPIb/IX, and GPIa/IIa were detected in 13, 10, and 8 patients, respectively. A response occurred in 7 of 7 patients without anti-GPIb/IX, but in only 3 of 10 patients with anti-GPIb/IX (p<0.01). There was no difference in the response rates in patients with or without anti-GPIIb/IIIa or anti-GPIa/IIa. We conclude that ITP patients with anti-GPIb/IX may be less responsive to IVIG.

The association between platelet autoantibody specificity and response to intravenous immunoglobulin G in the treatment of patients with immune thrombocytopenia.

This study was conducted to verify whether the response to high-dose intravenous immune globulin (IVIG) was related to the effect of splenectomy in chronic idiopathic thrombocytopenic purpura (ITP) patients. A total of 79 patients over 16 years of age were enrolled in this study. The response to the treatment was classified on the basis of the platelet count as no response (NR, 150 x 10(9)/l). The response was evaluated after the infusion of high-dose IVIG, within 2 weeks after splenectomy (immediate response), and during a follow-up period of more than 6 months after splenectomy (sustained response), respectively. 58 patients (73.4%) showed responses (CR or IR) to high-dose IVIG. After splenectomy, immediate responses were observed in 73 patients (92%). The response to high-dose IVIG had no relationship with the immediate response to splenectomy (P = 0.333). A follow-up evaluation was possible with 58 patients; 6 patients with NR in immediate responses did not show any response during the follow-up period, and 17 patients relapsed within 6 months after immediate responses, so 35 patients (60.3%) had sustained responses. Responders to IVIG had significantly higher sustained response rates to splenectomy than non-responders (62% vs. 38%, P = 0.001). These results indicate that the response to high-dose IVIG could be a valuable factor predicting the sustained response to splenectomy in chronic ITP patients.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/1096-8652%28200103%2966%3A3%3C197%3A%3AAID-AJH1044%3E3.0.CO%3B2-6

Response to high-dose intravenous immune globulin as a valuable factor predicting the effect of splenectomy in chronic idiopathic thrombocytopenic purpura patients

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Response to high-dose intravenous immune globulin as a valuable factor predicting the effect of splenectomy in chronic idiopathic thrombocytopenic purpura patients