Kojouri K, Vesely SK, Terrel DR et al.: Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess longterm platelet count responses, prediction of response, and surgical complications. Blood 104: 2623-34
Splenectomy has been a standard treatment for adult patients with idiopathic thrombocytopenic purpura (ITP) for more than 50 years. However, the durability of responses, the ability to predict who will respond, and the frequency of surgical complications with splenectomy all remain uncertain. To better interpret current knowledge we systematically identified and reviewed all 135 case series, 1966 to 2004, that described 15 or more consecutive patients who had splenectomy for ITP and that had data for 1 of these 3 outcomes. Complete response was defined as a normal platelet count following splenectomy and for the duration of follow-up with no additional treatment. Forty-seven case series reported complete response in 1731 (66%) of 2623 adult patients with follow-up for 1 to 153 months; complete response rates did not correlate with duration of follow-up (r = -0.103, P = .49). None of 12 preoperative characteristics that have been reported consistently predicted response to splenectomy. Mortality was 1.0% (48 of 4955 patients) with laparotomy and 0.2% (3 of 1301 patients) with laparoscopy. Complication rates were 12.9% (318 of 2465) with laparotomy and 9.6% (88 of 921 patients) with laparoscopic splenectomy. Although the risk of surgery is an important consideration, splenectomy provides a high frequency of durable responses for adult patients with ITP.
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"For patients with chronic ITP who failed to respond to corticosteroids or who can not tolerate corticosteroids, splenectomy is the second-line therapy in many centers . However, about 15–20% of patients do not respond well to splenectomy and among those who respond, 15–20% have relapse weeks, months, or even years later after splenectomy [8,9]. As the platelets in ITP are freshly out of the bone marrow and quite active, swift increases in platelet count may cause thrombosis . "
[Show abstract][Hide abstract]ABSTRACT: Immune trombocytopenia is an autoimmune disease characterised by abnormal platelet destruction. Corticosteroids and intravenous immune globulin are main first line treatment options. Splenectomy is a preferred second line treatment option for unresponsive patients at many centers. Secondary thrombocytosis is a possible complication awaiting this group of patients after splenectomy. Here, we present a case of myocardial infarction at a patient who had undergone splenectomy for ITP, which is a rare event seen with secondary thrombocytosis due to splenectomy.
"that reported in the literature. [7,8,15] Of those who achieved partial response, with the addition of glucocorticoids and/or azathioprine 86% (n=6) later progressed to CR, which was maintained for the duration of follow-up. This is in keeping with other studies showing that a partial response is also consistent with a favourable long-term outcome.  In our experience, the CR rate to splenectomy (platelet count >100 × 10 9 /L) and duration of response appear higher than that reported for rituximab and similar to that of the TPO agonists (with maintenance of treatment). [11,14] There was a 100% (n=4) mortality rate in the patients who showed no response following splenectomy; however"
[Show abstract][Hide abstract]ABSTRACT: New agents are being used as second-line treatment for immune thrombocytopenia (ITP) and have brought into question the relevance of splenectomy for steroid-resistant ITP.
We retrospectively analysed 73 patients who underwent splenectomy for ITP at our institution over an 11-year period. The median follow-up period was 25 months; patients with follow-up of <1 month were excluded. The outcomes of splenectomy were compared in HIV-positive v. HIV-negative patients.
The rate of complete response was 83%, and response was sustained for at least 1 year or until latest follow-up in 80% of patients. Twelve patients were HIV-positive. Splenectomy was laparoscopic in 43 patients (62%) with an overall 16% complication rate. The 90-day mortality rate was 1.38%. There was no statistically significant difference in response or complication rate in the HIV-positive patients. There was a statistically significant (p=0.003) poorer response to splenectomy in the patients with steroid-resistant ITP.
Splenectomy is effective and safe irrespective of HIV status and remains an appropriate second-line treatment for ITP. Further research is needed to corroborate our finding of lower response in patients who are steroid-resistant, as this might be a subgroup of patients who may benefit from thrombopoietin agonists as second line therapy.
"When the efficacy of splenectomy varied by 50% below the base case analysis, the differences between the cost-effectiveness ratios of sequences 1 and 2 and between sequences 1 and 3 increased from USD 1,158 to USD 3,707 and from USD 4,612 to USD 12,503, respectively (Figure 4 ). When the rituximab efficacy varied by 50% below the base case analysis, the differences between the cost-effectiveness ratios of sequences 1  14.4%  14.4%  26.8%  Emergency treatment rates (PL ≥ 30 × 10 9 /L) 0.4%  0.4%  0.4%  ― Mortality rate (PL < 30 × 10 9 /L) GR (%) × 4.2 + 0.02% † [1,3,21] GR (%) × 4.2 [1,21] GR (%) × 4.2 [1,21] GR (%) × 4.2 [1,21] Mortality rate (PL ≥ 30 × 10 9 /L) GR (%) × 1.8 [1,21] GR (%) × 1.8 [1,21] GR (%) × 1.8 [1,21] GR (%) × 1.8 [1,21] PL: platelet, GR: General mortality rate. References are given in brackets. "
[Show abstract][Hide abstract]ABSTRACT: Background
Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease in which the platelet count falls to <100¿×¿109/L. Corticosteroids are recommended as the first-line treatment, splenectomy is recommended as the second-line treatment, and thrombopoietin receptor agonists (TPO-RAs) and rituximab are recommended as the third-line treatments for ITP in Japanese ITP treatment guidelines. However, in Japan, rituximab is not eligible for reimbursement for the treatment of ITP. The cost-effectiveness of ITP treatment has not been investigated in Japan. Therefore, in this study, the cost-effectiveness of adding rituximab treatment to the existing treatments indicated for ITP in Japan, namely splenectomy and the TPO-RA romiplostim, was investigated based on the scenario that rituximab is eligible for reimbursement in Japan as a treatment for ITP.Methods
The efficacy endpoint was set as the number of years with a platelet count ¿30¿×¿109/L. The analysis was conducted from the healthcare payer¿s perspective. If the first treatment is ineffective or relapse occurs, then the patient is given the following treatment. The analyzed treatment order consisted of three patterns: splenectomy-romiplostim (sequence 1), splenectomy-romiplostim-rituximab (sequence 2), and splenectomy-rituximab-romiplostim (sequence 3). A Markov model was built for ITP, and the analysis period was set as 2 years. The discount rate was an annual rate of 2%.Sensitivity analyses of the efficacy of splenectomy, romiplostim, and rituximab; treatment cost; and romiplostim dose were performed.ResultsThe expected costs per patient over a 2-year period for sequences 1, 2, and 3 were USD 40,980, USD 39,822, and USD 33,551, respectively. The expected years with a platelet count ¿30¿×¿109/L for the three sequences were 1.75, 1.79, and 1.78 years, respectively. The sensitivity analyses illustrated that the results of the base case analysis were robust.Conclusions
Adding rituximab to standard treatment for ITP (sequences 2¿3) is less costly and marginally more effective than standard therapy in adults. According to the study results, if rituximab is reimbursed for the treatment of ITP in Japan, medical expenses are expected to decline.
Full-text · Article · Jan 2015 · BMC Health Services Research