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In primary chronic immune thrombocytopenia, long-term response to splenectomy, with 60% of patients enjoying a treatment-free life, is higher when compared with rituximab and similar to that with continuous thrombopoietin-receptor agonists (TPO-RA) administration. Splenectomy should continue to be offered to patients failing initial treatments in the absence of increased surgery-related risks. The higher lifelong safety concerns with splenectomy (increased risk of infection, shared in part with rituximab, and of thrombosis, in common with TPO-RA) and a mortality <1-2%, justify postponing surgery to the chronic phase, when spontaneous remissions are rarer. Patients failing initial treatment with corticosteroids/intravenous immunoglobulin may use TPO-RA (or rituximab in selected cases) as a bridge to surgery if they prefer to reconsider splenectomy later on, in case of no response, adverse effects or if sustained response after stopping TPO-RA is not attained. Special considerations apply in children aged ≤5 years, with splenectomy playing a marginal role. The recent approval of TPO-RA in children may represent a major advancement., (© 2018 John Wiley & Sons Ltd.)

Rationale: Refractory immune thrombocytopenic purpura (RITP) manifests as low platelet count, with a high risk of hemorrhage, treatment difficulty, and high mortality. Total hip arthroplasty (THA) in RITP is rarely reported. This study aimed to evaluate multiple THAs or revision total hip arthroplasties (RTHAs) in RITP., Patient Concerns: The male patient with RITP was 54-year-old patient and hospitalized on September 21, 2009, with the main complaint of bilateral hip pain after traveling for 2 weeks. The patient had a history of ITP for 16-years, with no response to hormone therapy (after adequate hormone therapy, platelet count persistently remained below 30 × 10/L). Two year prior to visit, the patient underwent splenectomy, and postoperative platelet persistently fluctuated around 10 to 20 × 10/L. The patient did not undergo regular reexaminations, and declined immunosuppressants., Diagnoses: Femoral neck fracture; Refractory immune thrombocytopenic purpura (RITP)., Interventions: A RITP patient with femoral neck fracture received 2 THAs and 1 RTHA. First THA indication was significant left dislocation of Garden III type. RTHA was performed following prosthetic loosening after left total hip arthroplasty. The second THA was prompted by non-healing of the old fracture, significant pain, and a low Harris score. Platelet count remained <20 × 10/L, and conventional drugs, splenectomy, and platelet transfusion had no effects. A large gamma-globulin dose was administered preoperatively. When platelet increased to 75 × 10/L, 2 THAs and 1 RTHA were successfully completed., Outcomes: Postsurgery, conventional management was applied; no severe complications occurred. The wound was well healed, with platelet count reduced to <15 × 10/L at hospital discharge. The patient recovered, with a Harris score >80 at 1 year postsurgery., Lessons: Extremely low platelet count is a contraindication of surgery. In this patient, preoperative platelet count was <100 × 10/L. Extended disease course and multiple operations lowered platelet count, and increased risk in surgery. However, high postoperative gamma-globulin dose impacted therapy, and all surgeries were successful, with no severe complications. The wound healed well, and the quality of life was significantly improved, demonstrating the feasibility and safety of this surgery. Multiple THA or RTHA surgeries are feasible and safe for RITP patients.

The recent large decrease in splenectomy use for chronic immune thrombocytopenia (ITP) is partly due to still-unsolved questions about long-term safety. We performed the first single-center exposed/unexposed cohort study evaluating the long-term incidence of splenectomy complications in patients with primary ITP. Overall, 83 patients who underwent splenectomy more than 10 years ago (exposed) were matched with 83 nonsplenectomized patients (unexposed) on the date of ITP diagnosis ±5 years, age and gender. After a median follow-up of 192 months (range 0.5-528), 43 patients (52%) achieved overall response after splenectomy. Splenectomized patients experienced more venous thromboembolism (VTE) than controls (n = 13 vs n = 2, P = 0.005). On multivariate analysis, splenectomy was an independent risk factor of VTE (hazard ratio = 4.006, P = 0.032 [95% confidence interval: 1.13-14.21]). Splenectomized patients presented more severe infections on long-term follow-up: all required hospitalization, and 5/26 (19%) infections led to severe sepsis or septic shock and to death for 3 cases (none in controls). However, the incidence of malignancy was similar in both groups, as was cardiovascular risk, which appeared to be related more to ITP than splenectomy. Finally, splenectomy did not significantly decrease overall survival. Despite the risk of thrombosis and severe sepsis, splenectomy remains an effective and curative treatment for ITP., Competing Interests: M. Michel has served as a consultant for Amgen and Glaxo-Smith Kline and received research support from Roche, participated on advisory boards and/or as a speaker at medical education events supported by Amgen and declares no competing financial interests. B. Godeau has served as a consultant for Amgen and GSK and received research support from Roche, participated on advisory boards and/or as a speaker at medical education events supported by Amgen, Roche, and GSK and declares no competing financial interests. The other authors declare no competing financial interests.

Intravascular lymphoma (IVL) is a rare form of B-cell lymphoma. We encountered a rare case of IVL diagnosed in an explanted liver. A 49-year-old man visited a clinic with high fever. Because of elevated liver function, he was diagnosed with acute liver failure. Deceased donor liver transplantation (LT) was performed 16 days after admission. The post-transplantation course was uneventful until IVL was reported in the explanted liver on postoperative day (POD) 21. Rituximab was administered on POD 27, and rituximab-cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone (R-CHOP) treatment administered on POD 38. The R-CHOP treatment was repeated for eight cycles, and the patient remains free of recurrence 1 year post-transplantation. Although systemic lymphoma is a contraindication to transplantation, our experience indicates that IVL can be successfully treated by the administration of prompt chemotherapy after LT for fulminant hepatitis., (© 2015 Steunstichting ESOT.)

Background: Obesity is still considered a relative contraindication to laparoscopic splenectomy (LS)., Methods: All patients undergoing LS at our institution were classified as obese or nonobese (group A, body mass index [BMI] >30; group B, BMI <30). Primary end points included conversion rate, operative complications, length of stay, operative time, and estimated blood loss (EBL)., Results: Three hundred seventy patients who underwent LS were included. Baseline characteristics were similar in groups A (n = 127; mean BMI, 36.2 ± 6.9 kg/m²) and B (n = 243; mean BMI, 24.6 ± 2.9 kg/m²). Conversion rates and overall morbidity were similar in both groups (9% vs 11% for conversion to open procedures, P = .621; 16% vs 16% for morbidity rates, P = .940). Length of hospital stay and EBL were also comparable (P = .643 and P = .544, respectively). Mean operative time was significantly increased in the obese group on multivariate analysis (170 vs 151 minutes, P = .021)., Conclusions: Obesity does not adversely affect outcomes after LS. The laparoscopic approach is the optimal technique for splenectomy regardless of the patient's weight., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Patients with immune thrombocytopenia (ITP) who relapse after an initial trial of corticosteroid treatment present a therapeutic challenge. Current guidelines recommend consideration of splenectomy, despite the known risks associated with surgery and the postsplenectomy state. To better define these risks, we identified a cohort of 9976 patients with ITP, 1762 of whom underwent splenectomy. The cumulative incidence of abdominal venous thromboembolism (AbVTE) was 1.6% compared with 1% in patients who did not undergo splenectomy; venous thromboembolism (VTE) (deep venous thrombosis and pulmonary embolus) after splenectomy was 4.3% compared with 1.7% in patients who did not undergo splenectomy. There was increased risk of AbVTE early (<90 days; hazard ratio [HR] 5.4 [confidence interval (CI), 2.3-12.5]), but not late (≥90 days; HR 1.5 [CI, 0.9-2.6]) after splenectomy. There was increased risk of VTE both early (HR 5.2 [CI, 3.2-8.5]) and late (HR 2.7 [CI, 1.9-3.8]) after splenectomy. The cumulative incidence of sepsis was 11.1% among the ITP patients who underwent splenectomy and 10.1% among the patients who did not. Splenectomy was associated with a higher adjusted risk of sepsis, both early (HR 3.3 [CI, 2.4-4.6]) and late (HR 1.6 or 3.1, depending on comorbidities). We conclude that ITP patients post splenectomy are at increased risk for AbVTE, VTE, and sepsis.

Background: Post-splenectomy infection has a mortality rate of up to 70%. Previously we have published data confirming the poor adherence to best practice guidelines with relation to management of the asplenic patient. A defined protocol of care was established, staff education commenced and a 'patient information leaflet' made available., Aim: To ascertain whether management of the asplenic patient has improved since the implementation of a structured programme of care., Method: Retrospective chart review of all splenectomies performed in Beaumont Hospital between 2002 and 2008., Results: Overall, 75.9% of patients were documented as having received the recommended vaccinations. Of these, 48.7% were not timed according to recommended guidelines. Prophylactic antibiotics were documented as prescribed in all but five patients discharged., Conclusions: These results demonstrate an improvement in post-splenectomy care between 2002 and 2008. However, further improvements are necessary.

Background: Idiopathic thrombocytopenic purpura (ITP) in adults has a chronic course and may necessitate splenectomy. The current study was undertaken to study the systemic thromboembolic complications of laparoscopic splenectomy (LS) versus open splenectomy (OS) in patients with ITP at two large referral hospitals., Patients and Methods: We conducted a retrospective analysis of 49 patients who underwent splenectomy (21 LS and 28 OS) for primary/relapsing refractory ITP between June 1995 and November 2004. Clinically and/or radiologically confirmed deep venous thrombosis (DVT) and/or pulmonary embolism (PE) were assessed within 2 weeks before and after splenectomy. None had prophylactic anticoagulants immediately after surgery. Follow up of those who developed complications continued for at least 2 additional years to assess for contributing factors that may have been masked at the time of occurrence., Results: Two (9.5%) LS group had acute PE within 5 days of LS and their platelet count reached 500A103/I(1/4)L within 4 days and 1000A103/I(1/4)L within 7 days after surgery. Three conversions to OS occurred; none had VTE. DVT occurred in 3 patients (10.7%) in the OS group; none were life threatening. There were no deaths., Conclusion: Life-threatening venous thromboembolic events are serious complications after LS and OS for ITP patients if prophylactic anticoagulants are not administered. Patients at risk are those who both have an exponential rise of the platelet count, although factors other than the platelet count may be contributing in OS. Postsplenectomy, ITP should be considered as a thrombophilic condition and studies of additional measures to prevent such events are warranted.

Background: Idiopathic thrombocytopenic purpura (ITP) comprises approximately 8% of all haemorrhagic diseases. Typical findings are a very low platelet count which manifests as petechial bleeding. Therapy consists of medication and removal of the spleen if conservative therapy fails., Patients and Methods: Between 1988 and 1999, 47 patients with ITP were splenectomized in our surgical department. We examine the postoperative development of platelet counts and long-term results in 33 of these patients., Results: After splenectomy, more then 75% of our patients had normal platelet counts. In long-term examination, 58% remained in stable condition with normal platelet counts. Retrospectively we tried to identify preoperative clinical features that could predict the long-term outcome of splenectomy in ITP but were unable to find reliable factors., Conclusion: Idiopathic thrombocytopenic purpura can be treated by surgical means but should be considered only when conservative treatment has failed. The long-term outcome of splenectomy is not predictable. Reliable predictive factors have to be identified through further research.

Thromboembolic complications following splenectomy for hematologic diseases occur in up to 10% of patients and may range from portal vein thrombosis (PVT) to pulmonary embolism (PE) and deep vein thrombosis (DVT). Up to now there exist no recommendations for the duration and intensity of prophylactic anticoagulation, which usually follows local institutional protocols. We report on three consecutive patients with severe portal vein thrombosis and/or pulmonary embolism--one with fatal outcome--7 to 35 days after splenectomy for autoimmune hemolytic anemia, immunothrombocytopenia, and indolent lymphoma, respectively. Incidence and pathophysiology of thromboembolic events (TE) in this patient group as well as prophylactic anticoagulation will be discussed, including a review of the current literature on this topic., (Copyright 2004 Wiley-Liss, Inc.)

Aim of the Study: Was to evaluate retrospectively the outcomes and efficacy of the laparoscopic splenectomies for ITP patients, performed at our institution over a period of 7 years and to compare these results with those after open splenectomies., Patients and Methods: We collected and analyzed data of 22 consecutive adult patients with ITP who underwent either laparoscopic (LS gr., n = 9) or open (OS gr., n = 13) splenectomy at Hospital of Kaunas University of Medicine between the years 1996 and 2002. The indications for splenectomy in these patients were unsuccessful treatment with corticosteroids or other medications and/or the requirement of high dosages of steroids for prolonged periods of time to maintain platelet count > 50 G/L before operation. Prior to surgery, all patients were treated with corticosteroids and/or intravenous immunoglobulin to raise the platelet count and to minimize the risk of intraoperative bleeding. The efficacy of the operation was evaluated by counting platelets one day before surgery and on the first and fifth postoperative day. Data chosen for analysis included age, gender, weight, height, American Society of Anaesthesiologists (ASA) score, number of converted patients, estimated blood loss during operation, operating time, postoperative secretion through the drains, morbidity, mortality and postoperative hospital stay., Results: There were no significant differences between LS and OS groups according patients age, weight, height, gender and ASA score. The mean operative time was 138.8 +/- 50.1 min in LS group and was significantly longer than operative time in OS group (102.3 +/- 21.3 min). One patient was converted to open splenectomy because of severe bleeding from splenic hilum. Postoperative complications occurred in one patient from each group. The mean intraoperative blood loss was 460 +/- 125 ml in LS group and 510 +/- 140 ml in OS group (p > 0.05). Postoperative secretion through the drains and postoperative secretion time in LS group was significantly lower and shorter than in OS group. Postoperative hospital stay in LS group (5 +/- 1.1 days) was significantly shorter than in OS group (8 +/- 1.4 days). After splenectomy, there was an immediate increase in the platelet count of all patients in both groups. Between the day before surgery and the first postoperative day, the mean platelet count rose significantly from 75 +/- 57.0 G/L to 117 +/- 84.2 G/L in LS group and from 64 +/- 60.1 G/L to 122 +/- 79.3 G/L in OS group. Between the first postoperative day and the fifth postoperative day, the mean platelet count also rose significantly in both groups: from 117 +/- 84.2 G/L to 259 +/- 151.0 G/L in LS group and from 122 +/- 79.3 G/L to 258 +/- 158.4 G/L in OS group. In the immediate postoperative period (five days after operation), all LS group and OS group patients responded to the splenectomy., Conclusions: Laparoscopic or open splenectomy are equally efficacious in patients with ITP, with an immediate response rate of 100 % in our study. Our study results show that open splenectomy appears superior to laparoscopic procedure in terms of shorter operative time. Laparoscopic splenectomy appears superior to open procedure in terms of postoperative hospital stay, postoperative drainage time, less postoperative secretion through the drains. These two approaches are similar with regard to blood loss during operations and the rate of postoperative complications.

Portal vein thrombosis (PVT) is a complication of hepatic disease and a potentially lethal complication of splenectomy. The reported incidence of this complication is low (approximately 1%). However, its true incidence may have been underestimated due to difficulty in making the diagnosis. Herein we report the case of a 19 year-old woman who presented with a 2-year history of idiopathic thrombocytopenic purpura (ITP). Because she had become refractory to medical therapy, she underwent laparoscopic splenectomy. She was discharged on postoperative day 2 after an uncomplicated procedure. She did well, complaining only of mild backache, until postoperative day 21, when she presented with nausea, vomiting, and leukocytosis. CT showed PVT and superior mesenteric vein thrombosis. Despite heparin and fluid administration, her condition worsened. At laparotomy, she had diffuse small bowel edema and congestion. At a second-look procedure 24 h later, nearly all her jejunum and ileum were necrotic. After three procedures, she was left with 45 cm of proximal and 10 cm of distal small bowel. Bowel continuity was restored 8 weeks later. She continued on warfarin anticoagulation therapy for 1 year. Postsplenectomy PVT is most often seen following splenectomy for myeloproliferative disorders and almost never after trauma. The large splenic vein stump and the hypercoagulable state in patients with splenomegaly are thought to be contributory. The presentation of PVT is vague, without defining signs or symptoms. Color-flow Doppler and contrast-enhanced CT scans are the best methods for the nonoperative diagnosis of PVT. Aggressive thrombolysis offers the best hope for clot lysis and maintenance of bowel viability. Even vague symptoms must be considered seriously following splenectomy.

We report a case of 58-year-old man who had repeated cardiac arrests on the first post-operative day. The patient underwent splenectomy due to ITP (idiopathic thrombocytopenic purpura). He also had diabetes mellitus and nephrotic syndrome. There was no abnormal finding at the preoperative examination, except bleeding time of 6 minutes. The operation was finished without complications under general anesthesia. Midnight on the day of surgery, the first cardiac arrest occurred, and lasted for about 10 seconds. He recovered soon from the incident, but at 6 o'clock next morning, he developed severe bradycardia and cardiac arrest. He recovered again, but around 10 o'clock, he developed bradycardia and arrest again, and fell into a fit of convulsions and lost his consciousness. Again he recovered soon and no bradycardia and cardiac arrest occurred after this episode. Two years later, he was scheduled for vitrectomy due to diabetic retinosis. There were a few PACs and PVCs in his Holter-ECG, but no typical bradycardia and ST changes. During the operation, we injected atropine sulfate, dopamine hydrochloride and bucladesine sodium to increase his heart rate above 60 per minute. The operation was finished smoothly and there was no trouble perioperatively. A year later, he also underwent bilateral cataract extraction under local anesthesia without any troubles.

The technical feasibility of laparoscopic splenectomy (LS) has been recently established. However, data regarding the efficacy of the procedure with long-term follow-up of patients with idiopathic thrombocytopenic purpura (ITP) are scarce. The objective of this study was to determine retrospectively the immediate efficacy and the long-term results of a standardized laparoscopic procedure applied to patients with ITP refractory to medical treatment. Laparoscopic splenectomy was performed in 35 patients for ITP between May 1993 and May 1998. The lateral approach was used in the last 27 patients. Data were recorded retrospectively on that group. Twenty-six patients (96%) underwent successful LS. Mean operative time for the laparoscopic procedure was 90 minutes. There were no postoperative deaths. Postoperative complications developed in three patients. Thrombocytopenia resolved after surgery in 93% of patients, but 7 patients (25%) experienced relapse during a mean 28-month follow-up. At present no patient needs medical therapy to maintain a normal platelet count. Laparoscopic splenectomy is feasible and safe in patients with ITP. Long-term results of LS for ITP are comparable to those achieved with open splenectomy.

A 58-year-old woman with idiopathic thrombocytopenic purpura required redo cardiac surgery of atrial septal defect closure, mitral annuloplasty, and tricuspid annuloplasty. Preoperative high-dose intravenous gamma-globulin and platelet transfusion after termination of cardiopulmonary bypass allowed successful redo cardiac surgery. Management of a patient with idiopathic thrombocytopenic purpura undergoing open heart surgery are discussed.

Mesenteric and portal vein thrombosis are rare complications of splenectomy. Their presentation depends on the underlying disease. It is most frequent in lymphoproliferative disorders but extremely rare in trauma. We describe a 22-year-old man and a 49-year-old woman who underwent splenectomy for idiopathic thrombocytopenic purpura and developed mesenteric and portal vein thromboses. Both were treated successfully with anticoagulants for 3 months until the thrombi regressed, as shown by CT scan. During a year of follow-up they were asymptomatic.

A 39-year-old female, who had splenectomy for idiopathic thrombocytopenic purpura (ITP) in 1988, was admitted to our hospital with high fever, headache, and loss of consciousness on November 29,1997. Neck stiffness and Kernig's sign were present. Examination of cerebrospinal fluid showed pleocytosis up to 506 cells/mm3 with 89% of polymorphonuclear cells and elevated protein to 1,135 mg/dl, and absence of glucose, Streptococcus pneumoniae phagocytosis was detected in the fluid. We diagnosed her as having pneumococcal meningitis as overwhelming postsplenectomy infection (OPSI) syndrome. After administration of dexamethasone (8 mg/day), cefotaxime (4 g/day), and ampicillin (6 g/day), she survived without any complications. Splenectomized patients have been recognized as immunocompromized hosts, and carry high morbidity and mortality risk from fulminant bacterial infections. Therefore, emergency treatment is important to reduce high mortality in such infections. We present an adult case of OPSI syndrome which occurred as pneumococcal meningitis, and we would like to emphasize the importance of prompt use of corticosteroids and high dose of sensitive antibiotics before DIC may occur during the course of illness.

We report the case of a female patient with thrombocytopenic purpura Werlhof. Splenectomy was followed by relapsing pneumococcal meningitis. Particularly, repetitive infections were caused by pneumococcus capsula type 13. This type is not included in the common 23-polyvalent vaccine matter. The necessity of splenectomy in autoimmunological diseases should be considered carefully in the individual case. Patients should be well informed of the high risk of infection after splenectomy and should undergo medical treatment immediately.

Background and Methods: Splenectomy is still a mainstay in the treatment of idiopathic thrombocytopenic purpura (ITP). Although the risk of fulminant sepsis after splenectomy in children and in adults has been known for a long time, the true prevalence of fulminant sepsis in adults splenectomized for ITP has still not been well established, despite many anecdotal reports. In this paper we have tried to estimate this risk by reviewing the available literature and by analyzing a large cohort of adults splenectomized for ITP at our Institute since 1970., Results: Two cases of fulminant sepsis (a 21-year-old man and a 24-year-old woman) were traced in a group of 133 splenectomized adults, with a crude incidence of 1.5% or, equivalently, of 0.17 cases per 100 patient-years. No case of sepsis occurred in a control group of 323 non splenectomized ITP adults. This estimation seems sufficiently precise for the large population analyzed and the duration and completeness of follow-up (1,126 patient-years, mean 101 months, 2 patients lost from follow-up), and it is in agreement with the estimations yielded by a literature review (1.8%)., Conclusions: The risk of fulminant sepsis seems to be a real hazard in adults splenectomized for ITP, and a conservative approach in recommending splenectomy seems advisable. Antipneumococcal vaccination should also be recommended.