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Background: Vaccines have been key in preventing COVID-19 infections and the AstraZeneca (AZ) vaccine has been widely used. However, increased rates of thromboembolic events were identified in recipients and, subsequently, a syndrome of vaccine-induced immune thrombotic thrombocytopaenia (VITT) was described whereby recipients presented with venous sinus thromboses, haemorrhagic infarctions, and - consequently - raised intracranial pressure. National guidance recommended decompressive craniectomies for refractory intracranial hypertension. We describe our neurosurgical experience in managing a cohort of patients with VITT., Clinical Presentation: Four patients were included (three females and one male); median age was 46 years. All patients presented with a constellation of headache, focal neurological deficit(s), altered consciousness, and/or seizure at a median 11 d post-vaccine. Pre-operatively, median GCS was 7 and the median platelet count was 28 × 10 9 /L. Mean craniectomy size was 13 cm × 10 cm. All four cases tested positive for anti-PF4 antibodies. Median length of stay was 9 d (range: 2-25). Of the four who were operated upon, two survived to hospital discharge, and one of these subsequently died at a peripheral hospital., Conclusions: VITT-related sinus thromboses and associated infarcts are rare complications of the AZ vaccine. Neurosurgical management involves treating intracranial hypertension however survival outcomes in our cohort were poor. In our series, decompression was performed in deteriorating patients however prophylactic decompression, in the presence of extensive venous sinus thrombosis, should be considered on a case-by-case basis. As vaccination programmes accelerate across the world, neurosurgeons are likely to be increasingly involved in managing intracranial hypertension in patients with VITT-related sinus thromboses.

Introduction: Splenectomy has been used as a diagnostic and therapeutic tool in the management of hematological diseases for many years. However, the emergence of new medical therapies has modified guidelines for many hematological diseases for which splenectomy was previously considered. We aimed to evaluate the evidence of a decrease in the hematological indications for splenectomy and the reasons and justifications for this change., Material and Methods: We conducted a single-center, retrospective analysis of patients who underwent laparoscopic splenectomy for hematological disease between January 2010 and December 2023. Patients were classified into four groups: 1 autoimmune and hemolytic diseases (HAD), (2) lymphomas, (3) myeloproliferative diseases (MPN), and (4) other splenic diseases. We recorded the annual incidence of splenectomy and the ratio of new medical cases, demographic and clinical data and surgical outcomes., Results: During the study period, 98 patients were referred for splenectomy. There was a significant progressive decrease in this surgical indication, particularly regarding HAD (p < 0.001). The indication for splenectomy for immune thrombocytopenic purpura (ITP) declined to zero despite an increase in the number of patients diagnosed with this disorder (p < 0.001). The pattern of decrease in AHAI and Evans syndrome was similar to that in ITP. The group of splenectomies due to lymphoma persisted consistently during the study period, as did the indication for splenectomy in the context of lymphoma treatment. Splenectomy due to massive splenomegaly secondary to MPN was indicated only in one patient. Splenectomies due to other causes were similarly distributed over the years., Conclusions: Our findings confirm a significant decrease in the indication for elective surgery for hematological diseases, mainly regarding autoimmune disease. The surgical community and surgical departments should be aware of this situation yet maintain the skills to adopt this technique both safely and efficiently., (© 2024. The Author(s).)

This study aimed to identify key proteomic analytes correlated with response to splenectomy in primary immune thrombocytopenia (ITP). Thirty-four patients were retrospectively collected in the training cohort and 26 were prospectively enrolled as validation cohort. Bone marrow biopsy samples of all participants were collected prior to the splenectomy. A total of 12 modules of proteins were identified by weighted gene co-expression network analysis (WGCNA) method in the developed cohort. The tan module positively correlated with megakaryocyte counts before splenectomy (r = 0.38, p = 0.027), and time to peak platelet level after splenectomy (r = 0.47, p = 0.005). The blue module significantly correlated with response to splenectomy (r = 0.37, p = 0.0031). KEGG pathways analysis found that the PI3K-Akt signalling pathway was predominantly enriched in the tan module, while ribosomal and spliceosome pathways were enriched in the blue module. Machine learning algorithm identified the optimal combination of biomarkers from the blue module in the training cohort, and importantly, cofilin-1 (CFL1) was independently confirmed in the validation cohort. The C-index of CFL1 was >0.7 in both cohorts. Our results highlight the use of bone marrow proteomics analysis for deriving key analytes that predict the response to splenectomy, warranting further exploration of plasma proteomics in this patient population., (© 2024 British Society for Haematology and John Wiley & Sons Ltd.)

Background: Splenectomy has been performed for various indications from haematological diseases to benign cysts and tumours, and for splenic traumatic injuries. However, there has been a steady decline in splenectomies in the last 20 years. The aim of this study is to establish the reasons behind this decline in splenectomy and to analyse them based on indication, type of splenectomy, and manner of approach (open, laparoscopic or robotic). Material and Methods: This is a retrospective study of a single centre experience of all the splenectomies, both total and partial, performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest) between 2002 and 2023. Only surgeries for primary splenic diseases were selected, splenic resections as part of other major operations were not included. Results: Between 2002 and 2023, 876 splenectomies were performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest). Most splenectomies (n=245) were performed for immune thrombocytopenic purpura (ITP), followed by benign tumours and cysts (n=136), lymphoma (n=119), hypersplenism due to cirrhosis (n=107) and microspherocytosis (n=95). Other indications included myelodysplastic syndrome (n=39), trauma (n=35), thalassemia (n=22), leukaemia (n=18) and also there were 60 splenectomies that were performed for hypersplenism of unknown cause. There were 795 total splenectomies (TS) and 81 partial splenectomies (PS). There was a decline in the number of splenectomies both TS and PS for all these indications, most notably in the case of ITP, microspherocytosis and hypersplenism due to cirrhosis with no splenectomies performed for these indications since 2020. Conclusion: With the development of new lines of treatment, advances in interventional radiology and in surgery with the spleen parenchyma sparing options, the need for total splenectomy has been greatly reduced which is reflected in the decline in the number of splenectomies performed in the last 20 years in our clinic., (Celsius.)

Background: Splenectomy is commonly used to treat refractory immune-mediated cytopenia, but there are no established factors that are associated with response to the procedure., Objectives: A cohort study was conducted to evaluate the hematologic and surgical outcomes of splenectomy in adult patients with immune cytopenias and identify preoperative factors associated with response., Methods: Data from the Cleveland Clinic Foundation for 1824 patients aged over 18 who underwent splenectomy from 2002 to 2020 were analyzed., Results: The study found that the most common indications for splenectomy were immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia, with a median age of 55 years and median time from diagnosis to splenectomy of 11 months. Hematologic response rates were 74% overall, with relapse in 12% of cases. Postsplenectomy discordant diagnoses were present in 13% of patients, associated with higher relapse rates. Surgery-related complications occurred in 12% of cases, whereas only 3% of patients died from disease complications. On univariate analysis, preoperative factors associated with splenectomy treatment failure were ≥3 lines of pharmacologic treatment, whereas isolated thrombocytopenia, primary ITP, and age ≤40 years had a strong association with response. The multivariable regression confirmed that treatment failure with multiple lines of medical therapy was associated with the failure to respond to splenectomy., Conclusion: Overall, the study demonstrates that splenectomy is an effective treatment option for immune-mediated cytopenias with a low complication rate., (© 2023 The Association for the Publication of the Journal of Internal Medicine.)

Despite the efficacy of splenectomy for chronic immune thrombocytopenia (ITP), its considerable failure rate and its possible related complications prove the need for further research into potential predictors of response. The platelet sequestration site determined by 111 In-labelled autologous platelet scintigraphy has been proposed to predict splenectomy outcome, but without standardisation in clinical practice. Here, we conducted a single-centre study by analysing a cohort of splenectomised patients with ITP in whom 111 In-scintigraphy was performed at La Paz University Hospital in Madrid to evaluate the predictive value of the platelet kinetic studies. We also studied other factors that could impact the splenectomy outcome, such as patient and platelet characteristics. A total of 51 patients were splenectomised, and 82.3% responded. The splenic sequestration pattern predicted a higher rate of complete response up to 12 months after splenectomy (p = 0.005), with 90% sensitivity and 77% specificity. Neither age, comorbidities, therapy lines nor previous response to them showed any association with response. Results from the platelet characteristics analysis revealed a significant loss of sialic acid in platelets from the non-responding patients compared with those who maintained a response (p = 0.0017). Our findings highlight the value of splenic sequestration as an independent predictor of splenectomy response., (© 2023 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)

Immune thrombocytopenic purpura (ITP) is a disorder caused by autoimmune antibodies which target glycoprotein IIb/IIIa complex or other platelet membrane antigens leading to platelet destruction. These platelets are then cleared by the spleen resulting in thrombocytopenia. Immune thrombocytopenic purpura affects about 1 to 6.4 cases in 100 000 children making it one of the most common causes of symptomatic thrombocytopenia in the pediatric population. It is rare that children or adolescents present with serious bleeding due to ITP. Common presentations include petechiae, bleeding gums, or bruising. Bleeding requiring hospitalization or transfusions is unusual and only occurs in approximately 5% of children. Even more uncommon is the presentation of severe bleeding complications requiring surgery for resolution. We present a case of a 17-year-old girl with acute ITP complicated by intraperitoneal hemorrhage and refractory thrombocytopenia due to ovarian cyst requiring oophorectomy., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Rituximab and thrombopoietin receptor agonists (TPO-RAs) have profoundly changed the management of immune thrombocytopenia (ITP) over the last 20 years. Even if most current guidelines put splenectomy, rituximab and TPO-RAs on the same treatment level, most clinicians and patients clearly prefer to postpone splenectomy and to multiply the lines of medical treatment before considering surgery. The management of ITP refractory to rituximab and TPO-RAs is challenging. Splenectomy is currently performed much less frequently because of a better knowledge of its complications, particularly severe late infections and deep vein thrombosis, and the inability to reliably predict its effectiveness. Furthermore, there is a reluctance to propose splenectomy when other treatments have been ineffective, based on the not well-documented risk that splenectomy could not be effective in such a case. The objective of this update was to review the most recent published data on the long-term tolerability and side effects of splenectomy and the predictors of response and efficacy, especially for patients exposed to multiple medical lines. This update can help physicians and patients with failure of multiple lines of therapy make an informed decision on the indication for splenectomy with the help of up-to-date data., (© 2023 The Author. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)

Splenectomy remains an effective treatment for refractory immune cytopenia (RIC), which encompasses immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Accessory spleens (AS) have been described without identifying specific risk factors. We retrospectively analyzed patients with RIC after splenectomy who underwent splenic scintigraphy (SS) at our institution. Seventy-one patients were included. Sixty-two patients had ITP, five had AIHA, and four had Evans syndrome. Seventy-five percent (n = 53) were women. Eleven patients (15.5%) had an AS detected by SS. A complete response (CR) to first-line steroids (odds ratio (OR) 5.75, 95% confidence interval (CI) 1.37-24.14, p = 0.017) and the absence of Howell-Jolly bodies (HJB) in peripheral blood smear (PBS) (OR 11.37, 95% CI 2.70-47.85, p = 0.001) were found to be risk factors. Patients with both elements had a higher rate of AS (83.3%) when compared to those with one or no factors (p < 0.001). Eight patients (73%) underwent an accessory splenectomy: seven (87.5%) achieved a CR, and none had perioperative complications. The presence of HJB in PBS changed from 25 to 87.5% after accessory splenectomy. We recommend the search for an AS via SS in patients with RIC due to ITP, who had a CR to corticosteroids and the absence of HJB in PBS. Accessory splenectomy is a safe and effective procedure., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Spontaneous spinal epidural hematoma (SSEH) can result from various etiologies with a variable degree of neurological deficits. Here, we describe a rare case of SEH secondary to immune thrombocytopenic purpura (ITP) in a child and review the literature of SSEH caused by ITP., Case Report: A 9-year-old female who presented with rapid neurological decline, including bowel and bladder incontinence and paraparesis. A SSEH was observed extending from C2 to T6, causing a mass effect on the spinal cord. Her platelet count was only 7000/µL. Multidisciplinary care was established with neurosurgery, pediatric hematology, and pediatric surgery. The patient was managed emergently with splenectomy and surgical evacuation, with multilevel laminectomy and laminoplasty for evacuation of the hematoma. After a short course of rehabilitation, the patient regained all neurological function., Conclusion: We report the first case of cervicothoracic SSEH secondary to ITP in a child managed with emergent splenectomy and surgical evacuation with multilevel lamoplasty. We also described the methods of timely diagnosis, urgent management, and overall prognosis of patients with this condition., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Purpose: Cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia (CVST-VITT) is an adverse drug reaction occurring after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. CVST-VITT patients often present with large intracerebral haemorrhages and a high proportion undergoes decompressive surgery. Clinical characteristics, therapeutic management and outcomes of CVST-VITT patients who underwent decompressive surgery are described and predictors of in-hospital mortality in these patients are explored., Methods: Data from an ongoing international registry of patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March 2021 and 10 May 2022, were used. Definite, probable and possible VITT cases, as defined by Pavord et al. (N Engl J Med 2021; 385: 1680-1689), were included., Results: Decompressive surgery was performed in 34/128 (27%) patients with CVST-VITT. In-hospital mortality was 22/34 (65%) in the surgical and 27/94 (29%) in the non-surgical group (p < 0.001). In all surgical cases, the cause of death was brain herniation. The highest mortality rates were found amongst patients with preoperative coma (17/18, 94% vs. 4/14, 29% in the non-comatose; p < 0.001) and bilaterally absent pupillary reflexes (7/7, 100% vs. 6/9, 67% with unilaterally reactive pupil, and 4/11, 36% with bilaterally reactive pupils; p = 0.023). Postoperative imaging revealed worsening of index haemorrhagic lesion in 19 (70%) patients and new haemorrhagic lesions in 16 (59%) patients. At a median follow-up of 6 months, 8/10 of surgical CVST-VITT who survived admission were functionally independent., Conclusions: Almost two-thirds of surgical CVST-VITT patients died during hospital admission. Preoperative coma and bilateral absence of pupillary responses were associated with higher mortality rates. Survivors often achieved functional independence., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

There are few reports of the long-term efficacy of splenectomy in children with immune thrombocytopenia (ITP). In a 33-year period, we performed splenectomies in 23 pediatric patients with ITP at a single institution in Japan. The age at surgery was 5-22 years with a median of 10 years. The follow-up period was 1-141 months with a median of 48 months. Before surgery, we confirmed the presence or absence of the accessory spleen by contrast-enhanced CT scan and we recommended vaccination with pneumococcal vaccine. Four patients underwent laparotomy before 1998, and 19 patients underwent laparoscopic surgery after 1999. Splenectomy showed high efficacy with a partial response rate of 83% and a complete response rate of 74%. Complete response was maintained in 70% of patients until the end of the observation period, and 91% were able to discontinue long-term management drugs such as steroids. No serious complications such as infectious diseases were observed. Although the number of cases here was small, the long-term efficacy and safety of splenectomy makes it a viable option in pediatric ITP despite the existence of newer therapeutic agents. Further research is necessary to compare the long-term efficacy and safety of splenectomy with new therapeutic agents., (© 2023. Japanese Society of Hematology.)

This article reports a case of spontaneous spinal subdural hematoma (SSDH) after brain surgery in a patient with immune thrombocytopenic purpura (ITP), reviews the relevant literature, and discusses the etiology, pathogenesis, and clinical features of SSDH in patients with ITP. A male patient in his early 50 s with an 8-year history of ITP and suffering from coexistent hemifacial spasm and trigeminal neuralgia underwent microvascular decompression in our department. His preoperative corrected platelet count was within the normal range. On postoperative day 2, the patient complained of acute low back pain and sciatica. Lumbar magnetic resonance imaging demonstrated an SSDH extending from L3 to L4 with a significantly decreased platelet count (30.0 × 10 9 /L). The pain was gradually relieved after 2 weeks of conservative treatment, and no neurological deficit occurred during the 1-year follow-up. Brain surgery may increase the risk of postoperative SSDH in patients with ITP. Clinicians planning brain surgery must conduct a rigorous assessment through detailed physical examination, laboratory tests, and medical history records and maintain perioperative platelet counts within the normal range to prevent various risks associated with spinal cord compression.

A 73-year-old male patient with postoperative recurrent rectal cancer developed thrombocytopenia after XELOX therapy. Thrombocytopenia persisted despite chemotherapy discontinuation;therefore, he was referred to our department for further evaluation. Bone marrow specimen examination revealed increased immature megakaryocytes and blood test results revealed elevated platelet-associated immunoglobulin G (PA-IgG) levels, leading to immune thrombocytopenic purpura diagnosis. His platelet count recovered after prednisolone therapy. Eltrombopag treatment was introduced considering thrombocytopenia secondary to chemotherapy resumption for rectal cancer. FOLFIRI therapy was continued without platelet count reduction, and PA-IgG levels decreased over time. The patient continued chemotherapy with eltrombopag and achieved a complete treatment response.

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a platelet count of less than 100 x 109 /L, resulting from antibody-mediated platelet destruction. Treatment for ITP typically involves steroids, and intravenous immunoglobulins (IVIG) can be added. Splenectomy is performed in cases with refractory ITP. Rituximab can suppress immunity but has limited efficacy in ITP cases. Herein, we present a rare case of a 30-year-old female who was first diagnosed with ITP and underwent a splenectomy two years later. However, seven years after surgery, she was presented with symptoms of ITP. A splenic scan showed an accessory spleen in the spleen bed, for which she underwent accessory spleen removal surgery. Her laboratory tests three days post-operation showed a rise in platelet count and hence was discharged a few days later. The patient had recurrent attacks of ITP even after the removal of the normal and accessory spleen, suggesting that accessory spleen removal may not always be an effective treatment for ITP. The patient eventually died. While splenectomy is a common treatment for ITP, it may not always be effective in all cases, and other treatments such as bone marrow transplantation may be necessary.

Background: Laparoscopic splenectomy (LS) is considered the gold standard treatment in adults with idiopathic thrombocytopenic purpura (ITP) refractory to medical therapy. However, the retrieval of the spleen in LS is still a technical challenge, despite the use of various commercial retrieval bags. This study reports the feasibility and reliability of using a saline bag for spleen retrieval in a reduced port splenectomy., Methods: Between 2007 and 2020, 55 consecutive patients underwent LS for ITP. Data were collected retrospectively. To retrieve the spleen, a 1 liter sterile saline bag was used., Results: Fifty-five patients underwent LS. There was only one complication related to the saline bag: an iatrogenic ileal injury during the morselization process., Conclusion: One-liter saline bag is feasible, and widely available. No additional instruments or cost is required and there is no need to extend the wound for spleen retrieval during LS., Key Words: Idiopathic thrombocytopenic purpura, Laparoscopic surgery, Splenectomy, Techniques.

Major options for second-line therapy in adults with chronic immune thrombocytopenia (ITP) include splenectomy, rituximab, and thrombopoietin receptor agonists (TRAs). The American Society of Hematology guidelines recommend rituximab over splenectomy, TRAs over rituximab, and splenectomy or TRAs while noting a lack of evidence on the cost-effectiveness of these therapies. Using prospective, observational, and meta-analytic data, we performed the first cost-effectiveness analysis of second-line therapies in chronic ITP, from the perspective of the U.S. health system. Over a 20-year time-horizon, our six-strategy Markov model shows that a strategy incorporating early splenectomy, an approach at odds with current guidelines and clinical practice, is the cost-effective strategy. All four strategies utilizing TRAs in the first or second position cost over $1 million per quality-adjusted life-year, as compared to strategies involving early use of splenectomy and rituximab. In a probabilistic sensitivity analysis, early use of splenectomy and rituximab in either order was favored in 100% of 10 000 iterations. The annual cost of TRAs would have to decrease over 80% to begin to become cost-effective in any early TRA strategy. Our data indicate that effectiveness of early TRA and late TRA strategies is similar with the cost significantly greater with early TRA strategies. Contrary to current practice trends and guidelines, early use of splenectomy and rituximab, rather than TRAs, constitutes cost-effective treatment in adults with chronic ITP., (© 2022 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.)

Background: We sought to identify factors associated with 30-day morbidity, and their impact on 30-day mortality, among patients undergoing splenectomy for immune thrombocytopenic purpura (ITP)., Methods: Using the ACS-NSQIP database, patients undergoing splenectomy for ITP were identified (2005-2019), and those with and without postoperative complications within 30 days of surgery were compared., Results: Of 2483 patients evaluated, 280 (11.3%) developed 30-day morbidity: infection (n= 145 [5.8%]), venous thromboembolism (n = 71 [2.9%]), acute renal failure (n = 7 [0.3%]), respiratory failure (n = 40 [1.6%]), cardiac arrest/myocardial infarction (n = 16 [0.6%]), cerebrovascular accident (n = 4 [0.2%]), or postoperative blood transfusion (n = 62 [2.5%]). Risk-factors for 30-day morbidity included age ≥50 years (odds ratio [OR] 1.50, p = 0.020), body mass index ≥30 kg/m 2 (OR 1.45, p = 0.023), functional dependence (OR 2.90, p = 0.009), preoperative albumin <3.5 g/dL (OR 2.10, p < 0.001), preoperative platelets <30 000/μL (OR 1.54, p = 0.020), open surgical approach (OR 2.32, p < 0.001), and inpatient status before surgery (OR 1.85, p = 0.040). Among patients at low-risk for 30-day morbidity (no risk-factors present), the 30-day morbidity rate was 5.0% versus 41.5% for ≥5 risk-factors (p < 0.001). Thirty-day mortality was 1.2%., Conclusions: Thirty-day morbidity and mortality are low with splenectomy for ITP. Select patients have particularly low perioperative risk and may benefit from early splenectomy if initial medical therapy fails., (© 2022 Wiley Periodicals LLC.)

Background: Splenectomy is an effective intervention in primary immune thrombocytopenia (ITP). Attempts to define pre-clinical predictors of platelet response to splenectomy are inconsistent. Based on international studies defining the likelihood of platelet response using platelet sequestration, patients with relapsed/refractory ITP being considered for splenectomy at a regional Australian hospital were assessed with 111 indium-labelled autologous platelet sequestration (ILAPS) studies., Aims: To audit the use of ILAPS in an Australian setting and define its role in predicting response to splenectomy., Methods: A retrospective review of all patients referred for an ILAPS study at a regional hospital was performed. Results for each patient were expressed as an 'R' value (spleen/ liver uptake ratio) to quantify the platelet sequestration pattern and outcome post-splenectomy, based on platelet counts., Results: A total of 45 patients was identified: 13 underwent splenectomy and 32 were medically managed. Patients with favourable ILAPS scans (pure or predominant splenic sequestration) demonstrated a superior response post-splenectomy (100% overall response rate (ORR); 83.5% complete remission (CR)) compared with those with unfavourable ILAPS scans (mixed or pure hepatic sequestration) (71.4% ORR; 57.1% CR) over 12 months., Conclusions: The use of ILAPS in the Australian setting is feasible and this experience confirms larger international studies demonstrating its utility as a predictor of response to splenectomy in ITP. An unfavourable ILAPS scan could be considered a negative predictor of response prompting consideration for other emerging ITP treatments such as thrombopoietin-receptor agonists or B-cell depleting therapy such as Rituximab., (© 2021 Royal Australasian College of Physicians.)

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder caused by autoantibodies against platelet antigens resulting in platelet destruction and inhibition of platelet production. Occasionally, an inciting event such as a virus or vaccination can precipitate ITP. Several cases of ITP have been reported after the BTN162b2 (Pfizer-BioNTech) and mRNA-1273 (Moderna) SARS-CoV-2 (COVID-19) vaccines. All reported cases of post-vaccination ITP have resolved with medical therapy until this case.A man in his mid-20s developed bleeding from ITP 2 weeks after receiving the second dose of the Pfizer SARS-CoV-2 vaccine. All inpatient medical treatment attempts failed. On hospital day 40, a splenectomy was performed and he ultimately recovered and was discharged.Awareness of potential vaccination side effects is a fundamental element of refining and improving patient safety. This case illustrates that given the right circumstances, serious refractory ITP can develop in response to the second dose of the Pfizer SARS-CoV-2 vaccine., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

The impact of splenectomy on health-related quality of life (HRQoL) in patients with immune thrombocytopenia (ITP) remains scarcely explored. Therefore, we evaluated HRQoL with the 36-Item Short-Form Health Survey (SF-36) in an internal cohort of 69 chronic ITP patients, overall and by type of treatment. Of these patients, 26 patients were splenectomized, while other patients were treated medically with thrombopoietin-receptor agonists (TPO-RAs) or immunosuppressive treatment. We also compared HRQoL of the splenectomized patients (internal cohort) with an external cohort of 63 splenectomized ITP patients and the general population. The median follow-up was 10 years (range 1-20). Splenectomized patients had a worse overall HRQoL profile than those who received medical therapy either with TPO-RAs or other treatments (OT), with clinically meaningful differences registered in several domains. These included physical functioning (Δ = - 17.0 and Δ = - 15.2, for TPO-Ras and OT, respectively, p = 0.065), role physical (Δ = - 9.7 and Δ = - 13.8, p = 0.483), and bodily pain (Δ = - 14.2 and Δ = - 18.8, p = 0.053). Compared to the general population, both internal and external splenectomized cohorts had an impaired HRQoL profile. Further studies on HRQoL in splenectomized ITP patients are needed to better understand the long-term impact of this surgical procedure., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Objectives: Management of refractory immune thrombocytopaenia (ITP) can be challenging. Amifostine, a thiophosphate prodrug, induces megakaryocyte maturation. In 2010, Fan et al. published results for 21 Chinese splenectomized patients, aged 13-92, with steroid-refractory ITP. Nineteen patients (15 patients aged  >18 years) achieved remission 2 months post-amifostine. This is the first publication utilizing amifostine and rituximab in refractory ITP., Materials and Methods: At the Cairns Hospital in Australia, we identified five patients treated with amifostine and rituximab for refractory ITP. Amifostine IV 400 mg once daily was administered 5 days/week for 5 weeks as tolerated. Rituximab was administered concurrently with/preceding amifostine based on clinician preference. Data were obtained through medical records and follow-up serology up to 5 years post-amifostine was available., Results: Three cases demonstrated durable responses up to 5 years' follow-up. One patient initially achieved remission but relapsed 1-year post-amifostine. One patient who did not have a splenectomy prior to amifostine did not respond., Conclusion: Three out of five patients achieved durable responses with amifostine and rituximab. Although there is confounding by rituximab, given its established low durable response rate, it is likely that the excellent long-term responses achieved were a result of amifostine. Clinical trials with larger patient cohorts and further investigation are required to confirm the efficacy and mechanism of amifostine in ITP., (© 2021 International Society of Blood Transfusion.)

In humans, splenectomy is performed to treat many clinical disorders, including immune thrombocytopenia. However, the incidence of splenectomies for immune thrombocytopenia as a therapeutic has significantly declined over the past decade due to the availability of new therapies. Infection and sepsis as a result of splenectomies are well documented, but other long-term effects are not well characterized. Evidence suggests that persons who have had a prior splenectomy may be at an increased risk of vascular conditions. Also, elevated levels of cell-derived microparticles appear to contribute to an increased risk of thrombosis and cardiovascular disease. However, in vivo studies on the increased levels of microparticles following splenectomy are limited. In order to understand the effects of splenectomies, we developed a protocol for splenectomy in adult zebrafish. After anesthesia, the spleen was removed under a stereomicroscope after making an incision on the ventral side of the fish. The spleen was removed by pulling with forceps. The incision was closed by Vetbond tissue glue. Blood collected from both splenectomized zebrafish and those that underwent sham surgeries was immunolabeled with polyclonal antisera against αIIb, followed by flow cytometry. We observed elevated levels of thrombocytes and their microparticles in splenectomized zebrafish. Finally, by injecting αIIb antibody intravenously into zebrafish, we found the thrombocyte counts decreased, suggesting the fish developed immune thrombocytopenia like conditions, which were then reversed by splenectomy. In summary, the model developed here should be useful to study molecular changes due to splenectomy. Also, the zebrafish will be useful in modeling treatment of immune thrombocytopenia like conditions.

Background: There are some difficulties regarding the evaluation of the post-splenectomy state., Objective: The objective of the study is to compare the post-splenectomy blood changes of immune thrombocytopenia (ITP) patients with those of trauma patients, 1 month and ≥ 6 months after surgery., Methods: Medical records of patients, who had undergone total splenectomy for ITP and trauma at a tertiary center between January 2009 and December 2019, were retrospectively reviewed., Results: The current study included 52 patients, who had undergone splenectomy for ITP (57.7%), and trauma (42.3%). Splenectomy, irrespective of the indications, resulted in an increase in hemoglobin concentration, hematocrit, and platelet levels. Neutrophils were responsible for the preoperative leukocytosis in ITP patients, and neutrophilia was ameliorated by splenectomy and also withdrawal of the steroid therapy in some patients. Decreased neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio supported the finding that splenectomy ameliorated inflammation in ITP patients. Splenectomy resulted in a change in percentages of leukocytes in favor of basophils in ITP patients., Conclusions: Splenectomy, irrespective of the indications, resulted in an increase in hemoglobin concentration, hematocrit and platelet levels, lymphocyte, monocyte, and eosinophil counts. Splenectomy ameliorated inflammation in ITP patients and resulted in a change in percentages of leukocytes in favor of basophils., (Copyright: © 2022 Permanyer.)

Although splenectomy is still considered the most effective curative treatment for immune thrombocytopenia (ITP), its use has significantly declined in the last decade, especially since the approval of thrombopoietin receptor agonists (TPO-RAs). The main objective of the study was to determine whether splenectomy was still as effective nowadays, particularly for patients with failure to respond to TPO-RAs. Our secondary objective was to assess, among patients who relapsed after splenectomy, the pattern of response to treatments used before splenectomy. This multicenter retrospective study involved adults who underwent splenectomy for ITP in France from 2011 to 2020. Response status was defined according to international criteria. We included 185 patients, 100 (54.1%) and 135 (73.0%) patients had received TPO-RAs and/or rituximab before the splenectomy. The median follow-up after splenectomy was 39.2 months [16.5-63.0]. Overall, 144 (77.8%) patients had an initial response and 23 (12.4%) experienced relapse during follow-up, for an overall sustained response of 65.4%, similar to that observed in the pre-TPO-RA era. Among patients who received at least one TPO-RA or rituximab before splenectomy, 92/151 (60.9%) had a sustained response. Six of 13 (46%) patients with previous lack of response to both TPO-RAs and rituximab had a sustained response to splenectomy. Among patients with relapse after splenectomy, 13/21 (61.2%) patients responded to one TPO-RAs that failed before splenectomy. In conclusion, splenectomy is still a relevant option for treating adult primary ITP not responding to TPO-RAs and rituximab. Patients with lack of response or with relapse after splenectomy should be re-challenged with TPO-RAs., (© 2021 Wiley Periodicals LLC.)

Objectives: Although splenectomy has long been second-line option for immune thrombocytopenia (ITP) patients, an indicator that reliably predicts the efficacy of splenectomy is still being explored. We investigated the treatment outcomes of splenectomy as a second-line therapy for relapsed/refractory ITP according to first-line intravenous immunoglobulin (IVIG) responses., Methods: Fifty-two adult patients treated with splenectomy as second-line therapy for ITP between 2009 and 2019 were included, and they were classified according to first-line IVIG responses (no response to IVIG: nonresponders; only transient IVIG response shorter than 4 weeks: poor responders; IVIG response for a longer period; stable responders). The efficacy of splenectomy was analyzed in the three subgroups., Results: Of the 52 patients, 10 were IVIG nonresponders, 34 were poor responders, and the remaining 8 were stable responders. Response to splenectomy was observed in 50.0% of IVIG nonresponders, 94.1% of poor responders, and 100% of stable responders (p = 0.0030). Among the 45 patients who responded to splenectomy, 51.1% relapsed subsequently, and a significantly lower relapse rate was noted in the stable IVIG responders (12.5%, p = 0.0220) than in nonresponders (60.0%) and poor responders (59.4%)., Conclusions: First-line IVIG response is indicated as a useful predictive factor for response to splenectomy., (© 2022 The Author(s). Published by S. Karger AG, Basel.)

The laparoscopic splenectomy in pediatric patients is performed worldwide but often the disproportion between size of patients and size of organs requires an extra laparotomic access for spleen removal. The aim of the present study was to evaluate the safety and effectiveness of the Alexis ® system to retrieve the spleen without additional laparotomic access. The charts of all patients who underwent splenectomy at our center during the last 5 years were retrieved. In all the cases the Alexis ® system was placed in the umbilicus, thru which a 10 mm camera was inserted. Three additional 5 mm standard trocars were inserted. Seven patients, affected by spherocytosis (3), epidermoid cyst (2), idiopathic thrombocytopenic purpura (2) and thalassemia (1), underwent laparoscopic splenectomy at a median age of 10 years (range: 8-17). Median patients' weight was 32.5 kg (range: 25-71) and spleen size 15 cm (11-18). In all the cases, upon removal of the camera, the retrieval bag was inserted thru the umbilicus under direct view, the spleen retrieved, morcellated, and removed. No conversion nor enlargement of one of the ports nor an extra laparotomic access were required. The patients were discharged on the fifth post-operative day and the cosmetic results were excellent. Removal of the spleen can be safely performed without any additional laparotomy thru the Alexis ® system placed in the umbilicus. This system is effective also in case of major patient/organ size disproportion and the final cosmetic aspect is excellent., (© 2021. The Author(s).)

Background: Minimally invasive splenectomy (MIS) is increasingly favored for the treatment of benign and malignant diseases of the spleen over open access approaches. While many studies cite the superiority of MIS in terms of decreased morbidity and length of stay over a traditional open approach, the comparative effectiveness of specific technical and peri-operative approaches to MIS is unclear., Objective: To develop evidence-based guidelines that support clinicians, patients, and others in decisions on the peri-operative performance of MIS., Methods: A guidelines committee panel of the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES) including methodologists used the Grading of Recommendations Assessment, Development and Evaluation approach to grade the certainty of evidence and formulate recommendations., Results: Informed by a systematic review of the evidence, the panel agreed on eight recommendations for the peri-operative performance of MIS for adults and children in elective situations addressing six key questions., Conclusions: Conditional recommendations were made in favor of lateral positioning for non-hematologic disease, intra-operative platelet administration for patients with idiopathic thrombocytopenic purpura instead of preoperative administration, and the use of mechanical devices to control the splenic hilum. Further, a conditional recommendation was made against routine intra-operative drain placement., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Primary immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by isolated thrombocytopenia. While first-line treatments focus on inhibiting autoantibodies and platelet destruction, second- and third-line treatments include splenectomy and thrombopoietin receptor agonists. In this study, we aimed to compare the efficiency and toxicities of splenectomy and eltrombopag as second-line treatments in ITP., Materials and Methods: We retrospectively analyzed patients who were diagnosed with ITP and followed between 2015 and 2020. Patients who underwent splenectomy or received eltrombopag treatment as second-line or further therapy were included. For subgroup analyses, patients were further stratified according to whether they received eltrombopag in the second or third line of treatment., Results: There were 38 patients in the splenectomy group and 47 patients in the eltrombopag group. The mean age of patients in the splenectomy and eltrombopag groups was 43.2 and 50.5 years, respectively. Time to response was significantly shorter in the splenectomy arm (p=0.001). However, response rates at the 3 rd , 6 th , 12 th , and 24 th months did not exhibit a statistically significant difference between groups; nor did total duration of response and adverse events. Response rates at the 1 st , 3 rd , 6 th , 12 th , and 24 th months and the total duration of response did not exhibit a statistically significant difference between eltrombopag subgroups. Eltrombopag treatment was ceased for 20 patients after a median of 54.1 months (range: 1-151). Among them, 12 patients (60%) did not experience a loss of response., Conclusion: Comparing the splenectomy and eltrombopag arms, even though time to achieve response was in favor of the splenectomy group, this advantage disappeared when overall response rates and response rate at the 2 nd year were considered. Using eltrombopag in the second or third line of therapy does not yield any difference in terms of time to achieving response.

Immune thrombocytopenia (ITP) is an autoimmune-mediated disease characterized by decreased platelet counts. Cytokines play important roles in modulating the immune response and are involved in the pathogenesis of many autoimmune diseases. This study aimed at exploring the serum levels of a core set of cytokines that exert immune-regulatory functions in newly diagnosed ITP patients (both before and after treatment) and splenectomized ITP patients. Using the Bio-Plex suspension array system and ELISA, the serum levels of IL-10, IL-21, IL-27, IL-33, IL-35, IL-37, and TGF-β 1 were detected. The data showed that the serum levels of the immune regulatory cytokines IL-10, IL-35, and TGF-β 1 were significantly lower in newly diagnosed ITP patients. Decreased cytokine levels could be improved in patients with a complete response or a response after steroid-based treatment(s). The serum concentrations of TGF-β 1 were positively correlated with the platelet counts both before and after treatment. All the detected immune-regulatory cytokines, except IL-37, showed significantly higher levels in splenectomized ITP patients than pretreatment ITP patients and healthy controls. In conclusion, these data suggest that lower levels of immune-regulatory cytokines are involved in the pathogenesis of ITP and that there is a long-lasting overexpression of immune-regulatory cytokines in ITP patients with splenectomy., (©2021 Society for Leukocyte Biology.)

Background: Despite the fact that Laparoscopic splenectomy has become first choice for most cases of splenectomy in the developed world, open splenectomy is still being done in majority of the developing countries. There is no local data of our hospital on laparoscopic splenectomy., Methods: Cross sectional study of 103 patients with laparoscopic splenectomy from Feb, 2013 to Jan, 2018 done for benign conditions of spleen at Khyber Teaching Hospital, Peshawar. Consecutive non-probability sampling was done. Lateral approach was utilized. Patients' demographics, preoperative characteristics and intra / postoperative complications were collected on a proforma., Results: Mean age was (26) years. Two third (2/3) of patients were female. Major indication was ITP (Idiopathic Thrombocytopenic Purpura) followed by HS (Hereditary Spherocytosis). Total splenectomy was done in splenic cysts. In half of the cases, platelet and / or blood were transfused preoperatively as a measure of preoperative optimization. Intraoperative & postoperative complications occurred in (8%) which were managed conservatively. Conversion rate was (0%). Triple vaccination was done in almost all patients., Conclusions: Laparoscopic splenectomy is safe in experienced hands and should be the first choice in elective cases even in developing world. Local surgeons should train residents and junior surgeons to improve laparoscopic skills as training abroad is costly.

Introduction: Laparoscopic splenectomy (LS) has become the standard treatment for benign hematological disorders and hypersplenism. However, serious complications such as pancreatic fistula and portal venous thrombosis (PVT) sometimes occur. We investigated the clinical significance of splenic vessels and anatomical features in LS. Methods: Patient data were collected from 32 patients who underwent LS. The indications for LS were hypersplenism due to liver cirrhosis, idiopathic thrombocytopenic purpura, hereditary spherocytosis, and others. Close contact of pancreatic tail with splenic hilum, spleen volume, and diameters of splenic vessels were evaluated on computed tomography images. Results: Close contact of pancreatic tail with splenic hilum was recognized in 15 of the patients. The close contact was significantly associated with operation time ( P  = .038), spleen volume ( P  = .021), and spleen volume/body surface area (BSA) ratio ( P  = .001). In multivariate analysis, spleen volume/BSA ratio was an independent factor for close contact ( P  = .022). PVT occurred in 3 cirrhosis patients, and the diameter of the splenic vein (SV) was significantly associated with PVT as a result of multivariate analysis ( P  = .027). Conclusion: Close contact of the pancreatic tail with the splenic hilum may cause a longer operation time at LS and be associated with spleen volume/BSA ratio. A larger SV diameter in cirrhosis patients may be related to PVT after LS.

Splenectomy is one of the treatments of immune thrombocytopenia (ITP) with a high response rate. However, it is an irreversible procedure that can be associated with morbidity in this setting. Our aim was to study the trends of splenectomy in adults with ITP, and the factors associated with splenectomy and resource utilization during these hospitalizations. We used the National (Nationwide) Inpatient Sample (NIS) to identify hospitalizations for adult patients with a principal diagnosis of ITP between 2007 and 2017. The primary outcome was the splenectomy trend. Secondary outcomes were (1) incidence of ITP trend, (2) in-hospital mortality, length of stay, and total hospitalization costs after splenectomy trend, and (3) independent predictors of splenectomy, length of stay, and total hospitalization costs. A total of 36,141 hospitalizations for ITP were included in the study. The splenectomy rate declined over time (16% in 2007 to 8% in 2017, trend p < 0.01) and so did the in-hospital mortality after splenectomy. Of the independent predictors of splenectomy, the strongest was elective admissions (adjusted odds ratio [aOR]: 22.1, 95% confidence interval [CI]:17.8-27.3, P < 0.01), while recent hospitalization year, older age, and Black (compared to Caucasian) race were associated with lower odds of splenectomy. Splenectomy tends to occur during elective admissions in urban medical centers for patients with private insurance. Despite a stable ITP hospitalization rate over the past decade and despite listing splenectomy as a second-line option for management of ITP in major guidelines, splenectomy rates consistently declined over time.

Background: The purpose of this study is to compare the clinical efficacy of laparoscopic splenectomy (LS) and open splenectomy (OS) in the treatment of Idiopathic thrombocytopenic purpura., Methods: We systematically searched PubMed, Web of science, EMBASE, Clinicaltrials.gov, and Cochrane Central Register for studies (study published from July 1992-January 2020). This study analyzed the clinical effect of LS and OS on idiopathic thrombocytopenic purpur., Results: This study showed that compared with OS, the LS's Overall response (OR: 0.60, 95% confidence interval (CI): 0.23-1.59, P = .30), Complication (OR: 0.59, 95% CI: 0.18-1.94, P = .38), Accessory spleen(OR: 1.70, 95% CI: 0.98-2.98, P = .06), Wound infections (OR: 0.65, 95% CI: 0.26-1.59, P = .34), Pancreatic fistula (OR: 0.73, 95% CI: 0.16-3.30, P = .68), was no significant, the Operative time (weighted mean difference (WMD): 49.33, 95% CI: 36.29-62.37, P < .00001)was longer, and the Estimated blood loss (WMD: -172.59, 95% CI: -319.96 to -25.22, P = .02), Postoperative length of stay (WMD: -4.68, 95% CI: -7.75 to -1.62, P = .003)was less., Conclusions: The therapeutic effect of LS was the same as that of OS in Overall response Complication Accessory spleen, while The operative time was longer, the Estimated blood loss was less, and the postoperative length of stay was shorter., Competing Interests: The author states that there is no funding and conflicts of interest., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction : Three distinct phases are recognized in immune thrombocytopenia (ITP): newly diagnosed (≤3 months after diagnosis), persistent (>3-12 months after diagnosis), and chronic (>12 months). Several international guidelines/expert recommendations have been released in the past 2 years regarding the treatment of newly diagnosed/persistent ITP. Areas covered : Across the guidelines/expert recommendations, thrombopoietin receptor agonists (TPO-RAs), including romiplostim (the focus of this review), are recommended in newly diagnosed or persistent ITP for patients who fail to respond to corticosteroids or intravenous immunoglobulin (or where these are contraindicated). To identify data relating to romiplostim in adults with newly diagnosed or persistent ITP, we conducted a search of PubMed (with no time limit applied) and abstracts from 2019 EHA/ASH meetings using the term 'romiplostim.' Expert opinion : The findings from nine clinical trials, six real-world studies and ten case reports provide insight into the early use of romiplostim, which could help to reduce exposure to the adverse effects associated with prolonged corticosteroid use, as well as reduce the risk of severe bleeding. Additionally, given the durable responses observed in patients with newly diagnosed/persistent ITP, as well as the potential for treatment-free responses following discontinuation, romiplostim might help to avoid the need for subsequent treatment.

A 91-year-old woman visited a local hospital with the chief complaint of bloody stool. The patient was noted that her platelet count is 1,000/μL, so she was referred to our hospital. Also after admission, she had bloody stool continuously. Then lower gastrointestinal endoscopy was done and it indicated that the reason for these symptoms is cecum colon cancer (cT3N0M0). We decided to perform an operation. Before the operation, in order to improve her platelet count to 100,000/μL high dose intravenous immunoglobulin, steroid therapy and platelet transfusion had done. The operation is laparoscopic ileocecal resection and the amount of bleeding is 10 g. The postoperative course was uneventful, and her platelet count became within normal range by platelet transfusion for 4 days. Until latest follow-up she has neither recurrence of the cancer nor thrombocytopenia. This case suggests that appropriate treatments make it impossible laparoscopic surgery for cecum colon cancer with ITP perform safety and resection for cancers may contribute to improve ITP.

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease in which the number of platelets decreases due to auto-antibodies against platelets. We report that thoracic endovascular aortic repair (TEVAR) was successfully performed for a thoracic aortic aneurysm complicated by ITP. The patient was a man of 77 years of age. He had a history of splenectomy due to ITP. He was admitted to our hospital with an aneurysm of the aortic arch that enlarged to a maximum minor axis of 63 mm. An operation was planned. Because of ITP, it was judged that replacement of the aortic arch using a cardio-pulmonary pump would be associated with a high risk of bleeding. Thus, 2-debranching TEVAR was selected and performed with no hemorrhagic complications. He was discharged from the hospital on the 12th day after surgery. We believe that 2-debranching TEVAR is effective for reducing perioperative bleeding in patients with ITP.

Objectives: Immune thrombocytopenia (ITP) is an autoimmune disease with heterogeneous background. FCGR2C mutations were defined in one third of the patients but genetic players have not been fully elucidated yet. Although childhood ITP present as benign, ITP in adulthood is chronic disease with treatment challenges. This study aimed to focus on adult ITP patients using a whole genome genotyping that is valuable approach to identify the responsible genomic regions for the disease., Methods: Herein 24 adult primary-refractory for ITP patients were evaluated using HumanCytoSNP12BeadChip,Illumina. Forty-six age and sex matched healthy individuals, and ptients awith nonhematological conditions were analyzed as controls. Identified CNV regions were verified by qRTPCR. T-cell receptor beta and delta (TCRB/TCRG) clonality were assessed by heteroduplex analysis in mosaic cases., Results: Several CNV losses and gains were defined (losses:2q,7q,17q,19p, and gains: 1q,2p,3q,4q,7q,10q,12p,13q,14q,15q,17p,20q,21p,22q,Xp). Mosaic changes of different sizes (0.2-17.77Mb) were identified in five patients and three of them showed clonality. CNV regions that were unique to ITP patients were identified for the first time and among these genes, those related to immune regulation, and cellular trafficking were noteworthy. Conclusion: Identified CNV regions harbor several candidate genes, the functions of which might shed light on the pathogenesis of chronic ITP.

Childhood chronic immune thrombocytopenic purpura (cITP) is a rare disease. In severe cases, there is no evidence for the optimal therapeutic strategy. Our aim was to describe the real-life management of non-selected children with cITP at diagnosis. Since 2004, patients less than 18 years old with cITP have been enrolled in the national prospective cohort, OBS'CEREVANCE. From 1990 to 2014, in 29 centres, 392 children were diagnosed with cITP. With a median follow-up of six years (2·0-25), 45% did not need second-line therapy, and 55% (n = 217) received one or more second lines, mainly splenectomy (n = 108), hydroxychloroquine (n = 61), rituximab (n = 61) or azathioprine (n = 40). The overall five-year further second-line treatment-free survival was 56% [95% CI 49·5-64.1]. The use of splenectomy significantly decreased over time. Hydroxychloroquine was administered to children with positive antinuclear antibodies, more frequently older and girls, and reached 55% efficacy. None of the patients died. Ten years after the initial diagnosis, 55% of the 56 followed children had achieved complete remission. Children with cITP do not need second-line treatments in 45% of cases. Basing the treatment decision on the pathophysiological pathways is challenging, as illustrated by ITP patients with positive antinuclear antibodies treated with hydroxychloroquine., (© 2020 British Society for Haematology and John Wiley & Sons Ltd.)

Despite the publication in 2009 of a paper on 'terms and definitions of immune thrombocytopenia' (ITP), some unresolved issues remain and are reflected by the disagreement in the treatment suggested for primary ITP in adults. Considering that these disagreements could be ascribed to non-shared goals, we generated a 'consensus' on some terms, definitions, and assertions useful for classifying the different lines of treatment for primary ITP in adults according to their indications and goals. Agreement on the appropriateness of the single assertions was obtained by consensus for the following indicators: 1 . classification of four 'lines of therapy'; 2 . acceptance of the expression 'sequences of disease' for the indications of the respective four lines of treatment; 3 I . practicability of splenectomy; 3 Ib . acceptance, with only some exceptions, of a 'timing for elective splenectomy of 12 months'; and 4 a-d . 'goals of the four lines of therapy.' On the basis of the consensus, a classification of four lines of treatment for primary ITP in adults was produced. In our opinion, this classification, whose validity is not influenced by the recently published new guidelines of the American Society of Hematology (ASH) and reviews, could reduce the disagreement that still exists regarding the treatment of the disease.