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The treatment strategy of adult idiopathic thrombocytopenic purpura (ITP) in Japan had consisted of corticosteroids as the first-line option, splenectomy as the second-line option, and others as the third-line option, respectively, for a long time. However, thrombopoietin receptor agonists (TPO-RAs) have been widely adopted recently for corticosteroid-resistant ITP, and indications for rituximab have been extended to adult ITP in Japan, suggesting that ITP treatment is dramatically changing. In the "Reference guide for adult ITP treatments in Japan" revised in 2019, TPO-RAs and rituximab are equally recommended as second-line treatments alongside splenectomy. It is suggested to select from among the second-line treatments with careful consideration of not only their advantages and disadvantages but also aspects of the condition and situation of each patient such as any comorbidities or lifestyle factors. Moreover, since multiple effective therapeutic options are now available as second-line options, it is preferable to consider an early transition to second-line treatments for corticosteroid-refractory/dependent ITP patients.

Spontaneous improvement (SI) occurs more frequently in children with chronic immune thrombocytopenia (cITP) than in adults. It is generally accepted that, with the exception of splenectomy, conventional medical approaches for cITP do not change the natural course of the disease. Previous studies on pediatric cITP have reported prognostic factors associated with SI; however, it is important to know when such improvement occurs to enable optimal treatment strategies for cITP. Here, we report results of retrospective analysis of 56 consecutive pediatric patients with cITP at our institution. The median follow-up period after ITP diagnosis was 67 months (11-185 months). Of the 44 patients without splenectomy, 17 achieved SI at a median age of 8.5 years (2.3-16.5 years). The estimated incidence of SI was 24.6 ± 6.0 % at 36 months. In 16 of the 17 patients with SI, the recovery was achieved within 18 months from diagnosis, or at an age of less than 10 years, whereas among the 24 who did not achieve spontaneous improvement both at "an age of 10 years or more" and at "18 months or more from ITP diagnosis", only one recovered spontaneously. A treatment decision tree, including the indication for splenectomy, should be considered based on this watershed point.

Background: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder for which appropriate diagnostic treatments are uncertain. The response to splenectomy varies from 60% to 90%, and the remaining patients relapse and require further treatment. Therefore, it is important to predict the outcome of splenectomy before and after surgery. The objective of this study was to evaluate the efficacy of splenectomy in patients diagnosed with ITP., Materials and Methods: From 1988 to 2004, we splenectomized 32 patients with ITP; 17 underwent laparoscopic splenectomy (LS) and 15 underwent conventional open splenectomy (OS). For analysis, patients were separated retrospectively into two groups: the "responding group," those who showed good outcomes with splenectomy, and the "non-responding group," those who did not show good outcomes with splenectomy. Blood samples were examined before and immediately after surgery (day 0) and on postoperative days (POD) 1, 3, 5, and 7., Results: The median follow-up was 8.3 years (range: 1-16 years). The overall 5- and 10-year survival rates after splenectomy were 96.9% (one death). The responding group included 24 patients (75%), and the non-responding group included 7 (21.9%). Platelet counts in the responding group increased gradually until POD 7, and although platelet counts in the non-responding group were almost constant until POD 5, they subsequently decreased until POD 7. Average platelet counts in the responding and non-responding groups were 269 and 124 x 10(9)/l on POD 7, respectively (P < 0.05). The pre- to post-surgery ratio of platelet counts were almost the same as the result of the actual data. Platelet counts during the long-term follow-up for the responding and non-responding groups were related to those noted on discharge., Conclusions: A high platelet count on POD 7 was associated with a good response to splenectomy, but age at surgery, the time interval between diagnosis and splenectomy, and prior responses to corticosteroid were not. We suggest that long-term outcomes of splenectomy can easily be predicted by platelet counts on POD 7.