Case report: Fatal overwhelming post-splenectomy infection in a patient with metastatic angiosarcoma treated with immunotherapy.

A patient in his 40s with splenic angiosarcoma metastatic to the liver underwent splenectomy, chemotherapy, and partial hepatectomy before being treated on a clinical trial with CTLA4 and PD1 inhibitors. He had received pneumococcal and meningococcal vaccines post-splenectomy. On week 10, he developed grade 3 immune-related colitis, successfully treated with the anti-tumor necrosis factor-alpha inhibitor infliximab and steroids. After 4 cycles of treatment, scans showed partial response. He resumed anti-PD1 therapy, and 6 hours after the second dose of anti-PD1 he presented to the emergency room with hematemesis, hematochezia, hypotension, fever, and oxygen desaturation. Laboratory tests demonstrated acute renal failure and septicemia (Streptococcus pneumoniae). He died 12 hours after the anti-PD1 infusion from overwhelming post-splenectomy infection (OPSI). Autopsy demonstrated non-viable liver tumors among other findings. In conclusion, patients undergoing immunotherapy and with prior history of asplenia should be monitored closely for OPSI as they may be at increased risk.

Purpura fulminans due to Enterococcus cecorum in an asplenic patient.

Enterococcus cecorum was initially isolated from the intestine of poultry and is an uncommon cause of human infection. We report here what we believe to be the first case of overwhelming post-splenectomy infection (OPSI) with purpura fulminans due to Enterococcus cecorum in a 51-year-old man. As opposed to other enterococci, Enterococcus cecorum remains susceptible to third-generation cephalosporin which is the first line empirical antibiotic therapy for both patients with purpura fulminans and asplenic patients with sepsis. Despite adequate antibiotic therapy, evolution in the intensive care unit (ICU) was overwhelming with death occurring 10 h after ICU admission.

Immunization coverage among asplenic patients and strategies to increase vaccination compliance: a systematic review and meta-analysis.

BACKGROUND: Asplenic or splenectomized patients have a higher risk (ranging from 10 to 50-fold) than the general population of developing an overwhelming post-splenectomy infection (OPSI). Thus, they should receive specific vaccinations to prevent bacterial infections and influenza. The aim of this meta-analysis was to estimate vaccination coverage (VC) with the recommended vaccines among splenectomized patients; strategies recommended in those studies to improve VC worldwide are considered as well. RESEARCH DESIGN AND METHODS: Scopus, MEDLINE/PubMed, Google Scholar and ISI Web of Knowledge databases were searched. Research papers, short reports, reviews, and meta-analyses published between January 1, 2010 and July 18, 2020 were included; no geographic restrictions were included. Twenty-four studies were included in the meta-analysis. RESULTS: For anti-pneumococcal vaccination, coverage was 55.1% (95%CI = 41.0-69.2%), for anti-Hib 48.3% (95%CI = 34.3-52.3%), for anti-meningococcal C/ACYW135 33.7% (95%CI = 23.6-43.9%), for anti-meningococcal B 13.3% (95%CI = 7.0-19.5%) and for anti-influenza 53.2% (95%CI = 22.0-84.4%). Most studies determined a lack of adherence to international guidelines by healthcare workers and suggested the need to better educate health professionals in the management of post-splenectomy patients. CONCLUSIONS: The meta-analysis showed the suboptimal immunization coverage for the vaccines recommended for asplenic patients. Greater efforts must be made by public health professionals to increase VC in this group of patients at risk. Introducing specific prophylaxis protocols in the clinical routine seems to guarantee better immunization compliance in those patients.

Overwhelming Post-Splenectomy Infection Syndrome: Variability in Timing With Similar Presentation.

Overwhelming post-splenectomy infection (OPSI) syndrome is a rare and well-known entity that rapidly progresses with poor outcomes. Two patients underwent splenectomy after trauma and later presented with flu-like symptoms and thrombocytopenia, which then progressed to fulminant sepsis and death. The first patient had sepsis 20 days post-splenectomy, and the second patient underwent splenectomy 15 years before presentation. Both patients expired within 24 hours of the onset of symptoms. Even with no specific criteria for diagnosis, prompt identification of the overwhelming post-splenectomy infection is necessary; however, the prognosis is usually poor, even with aggressive treatment.

What are the Particularities of Splenic Surgery in Cirrhotic Patients?

The aim of this work was to review the entire literature on splenic surgery in cirrhotic patients in order to best define the surgical indications and their management specifics. A review of the international literature published between January 1995 and August 2015 was thus carried out.

Post-splenectomy Sepsis: A Review of the Literature.

The spleen is an intraperitoneal organ that performs vital hematological and immunological functions. It maintains both innate and adaptive immunity and protects the body from microbial infections. The removal of the spleen as a treatment method was initiated from the early 1500s for traumatic injuries, even before the physiology of spleen was properly understood. Splenectomy has therapeutic effects in many conditions such as sickle cell anemia, thalassemia, idiopathic thrombocytopenic purpura (ITP), Hodgkin's disease, and lymphoma. However, it increases the risk of infections and, in some cases, can lead to a case of severe sepsis known as overwhelming post-splenectomy infection (OPSI), which has a very high mortality rate. Encapsulated bacteria form a major proportion of the invading organisms, of which the most common is Streptococcus pneumoniae. OPSI is a medical emergency that requires prompt diagnosis (with blood cultures and sensitivity, blood glucose levels, renal function tests, and electrolyte levels) and management with fluid resuscitation along with immediate administration of empirical antimicrobials. OPSI can be prevented by educating patients, vaccination, and antibiotic prophylaxis. This article summarizes the anatomy and physiology of the spleen and highlights its important functions. It primarily focuses on the pathophysiology of OPSI, its current management, and prevention strategies.

Prevalence and distribution of functional splenic tissue after splenectomy.

BACKGROUND: Individuals splenectomised for trauma have lower infection rates than those splenectomised for other conditions. Residual functional splenic tissue (FST) after splenectomy may provide ongoing immunological protection. AIMS: To quantify the prevalence and volume of residual FST post-splenectomy using standard testing. METHODS: Splenectomised adults were recruited from the Spleen Australia clinical registry. Eligible individuals had been splenectomised at least 1 year prior to their visit and resided in Victoria. Splenic function was identified by evaluating Howell-Jolly bodies and IgM memory B cells. A 99m-Technetium-labelled, heat-denatured erythrocyte scintigraphic scan was performed if splenic function was detected. RESULTS: Initially, 75 splenectomised individuals (all cause) were recruited, with a median of 58 years of age and who were splenectomised a median of 14 years previously. The most common indications for splenectomy were trauma (30.7%) and haematological disease (28.0%). Scintigraphy identified FST in nine individuals (12.0%). Eight had been splenectomised for trauma. In this cohort, 34.8% of individuals splenectomised for trauma had residual FST. To explore our findings further, 45 additional individuals were recruited, predominately individuals splenectomised for trauma. Twenty-five individuals completed assessments by December 2018. An additional 11 individuals had FST, of whom 9 had been splenectomised for trauma. Overall, we identified 20 individuals with residual FST. Volumes ranged from 2.2 to 216.0 cc. We saw individuals with accessory spleens and splenotic nodules and an individual with both. Seventeen individuals had been splenectomised for trauma. CONCLUSIONS: Residual FST is commonly seen in individuals splenectomised for trauma. It can present in varying distributions and of varying volume. The clinical significance is unclear.

Post-splenectomy sepsis: preventative strategies, challenges, and solutions.

Removal of the spleen had already been established as a routine technique to treat splenic trauma and other diseases affecting the spleen before the anatomy, physiology, and function of the spleen were known in the mid-twentieth century. It is now widely accepted that the splenectomized individual is at increased risk for infection, in particular, overwhelming post-splenectomy infection (OPSI). OPSI is a syndrome of fulminant sepsis occurring in splenectomized (asplenic) or hyposplenic individuals that is associated with high mortality and morbidity. Poorly opsonized bacteria such as encapsulated bacteria, in particular, Streptococcus pneumoniae, are often implicated in sepsis. The spleen is a reticuloendothelial organ that facilitates opsonization and phagocytosis of pathogens, in addition to cellular maintenance. Splenectomy is associated with an impairment in immunoglobulin production, antibody-mediated clearance, and phagocytosis, leading to an increased risk of infection and sepsis. Early identification of the at-risk patient, early blood cultures prior to antibiotic administration, urgent blood smears and fast pathogen-detection tests, and sepsis bundles should be utilized in these patients. Prompt management and aggressive treatment can alter the course of disease in the at-risk splenectomized patient. Overwhelming post-splenectomy infection can be prevented through vaccination, chemoprophylaxis, and patient education. This article evaluates post-splenectomy sepsis by summarizing the anatomy and function of the spleen, physiological changes after splenectomy that predispose the splenectomized patient to infection, and current management and prevention strategies.

The non-coding RNome after splenectomy.

Splenectomy is a common surgical procedure performed in millions of people worldwide. Epidemiologic data show that splenectomy is followed by infectious (sepsis) and non-infectious complications, with unknown mechanisms. In order to explore the role of the non-coding transcripts involved in these complications, we analysed a panel of circulating microRNAs (miRNAs), which were previously reported to be deregulated in sepsis, in the plasma of splenectomized patients. MiR-223 was overexpressed immediately and late after splenectomy, while miR-146a was overexpressed immediately after splenectomy, returning latter to basal levels; and miR-16, miR-93, miR-26a and miR-26b were overexpressed only late after splenectomy, suggesting similarities with sepsis. We also explored the non-coding (nc)RNome of circulating peripheral blood leucocytes by performing a ncRNA full genome profiling. We observed a reorganization of the ncRNoma after splenectomy, characterized by up-regulation of miRNAs and down-regulation of transcribed pyknons (T-PYKs). Pathway analysis revealed that deregulated miRNAs control pathways involved in immunity, cancer and endothelial growth. We checked the expression of the ncRNAs in 15 immune cell types from healthy donors and observed that plasma miRNAs, cellular miRNAs and T-PYKs have a cell-specific expression pattern and are abundant in different types of immune cells. These findings suggest that the ncRNAs potentially regulate the immune changes observed after splenectomy.

A case report of septic shock syndrome caused by S. pneumoniae in an immunocompromised patient despite of vaccination.

BACKGROUND AND CASE PRESENTATION: We report a case of septic shock syndrome caused by Streptococcus pneumoniae in a patient who had undergone splenectomy due to an autoimmune lymphoproliferative syndrome (ALPS), which is characterized as a dysfunction of immunoregulation. Although the patient was vaccinated with a conjugated polysaccharide vaccine after the splenectomy, he was still susceptible to S. pneumoniae infection, because the isolated serovar (24F), a serovar long thought to be apathogenic, is not covered by any vaccine currently approved, neither a conjugated nor an unconjugated polysaccharide one. CONCLUSIONS: This case demonstrates that, due to presence of different serovars, also infections with bacteria against which patients are vaccinated have to be considered as differential diagnosis. Although vaccine development has extended the coverage of S. pneumoniae from 7 to 23 serovars within recent years, there is still demand for novel vaccines which can provide broader protection also against so-thought "apathogenic" strains, especially for groups at high risk.

Splenic Autotransplantation Is Always Valid after Splenectomy.

When splenectomy is performed, autotransplantation is the only method to preserve splenic function. The most frequently used technique for splenic autotransplantation in humans is the implantation of multiple sections of the splenic parenchyma into pouches created in the greater omentum. However, this technique of autotransplantation is associated with complications. For this reason, a technique in which only one 35-g slice of spleen is transplanted into the greater omentum but positioned within the native hypochondrium can be considered safe and useful for patients. Experimental studies continue to add valuable information to the ongoing research in the field of autotransplantation, providing a baseline for future studies in humans and adding arguments in favor of autotransplantation when the spleen cannot be preserved.

Partial Splenectomy in the treatment of an adult with ß thalassemia intermedia: A case report.

INTRODUCTION: Thalassemia is a common disease which treatment is often based on splenectomy. The risks associated with total splenectomy stimulated partial splenectomy as a potentially alternative therapy. CASE PRESENTATION: A 45 year-old female patient with long term follow-up for ß thalassemia intermedia started to develop signs of hypersplenism and iron overload. A partial splenectomy was performed and was observed a marked hematologic improvement while preserving the desired splenic function. DISCUSSION: Partial splenectomy proved to provide a persistent decrease in hemolytic rate while preserving the integrity of splenic phagocytic function, presenting itself as an effective alternative to total splenectomy. After being subjected to partial splenectomy, our patient experienced a sustained control of hemolysis and showed no signs of hypersplenism or iron overload. No splenic regrowth or infectious complications were observed. The major drawbacks of partial splenectomy are the increased risk of intra- and postoperative bleeding, splenic remnant torsion and splenic regrowth. CONCLUSION: Partial splenectomy is an alternative to total splenectomy for the treatment of adult ß Thalassemia intermedia patients avoiding the risks associated with total splenectomy.

The sword of Damocles for the splenectomised: death by OPSI.

The overwhelming post splenectomy infection (OPSI) in splenectomised patients is a rare but severe infection mostly caused by encapsulated bacteria. We analyse the case of a 65-year-old female patient who was presented with clinical and laboratory findings indicating gastroenteritis. Two years years before admission, the patient underwent a splenectomy for a two stage splenic rupture following resuscitation for pulmonary embolism. Immunisation of the patient was complete and timely. As a result of the unspecific clinical presentation, there was a delay in administration of antibiotics. However, administration of antibiotics induced a fulminant shock. The patient died 4 hours after attending the hospital due to a pneumococcal sepsis. The discussion highlights epidemiological and pathophysiological aspects and potential prevention strategies in the international context. Vaccination failed in our patient as the isolated pneumococcal strain (serogroup: 12F) is usually covered by the 23-valent pneumococcal polysaccharide vaccination (Pneumovax(®)). The case reported here indicates that there may be a potential benefit of prophylactic antibiotic treatment within the first 3 years after splenectomy for patients above the age of 65 years. Awareness of OPSI (prevention strategies, symptoms and treatment) among patients and their treating physicians is crucial for the improvement of prognosis. We partly address these issues in a standard operating procedure for the assessment of splenectomised patients in our departments of emergency medicine.

Overwhelming post splenectomy infection syndrome - review study.

The spleen has an abundance of lymphoid tissue, including splenic macrophages that attack encapsulated organisms. Overwhelming post-splenectomy infection (OPSI) is a serious disease that can progress from a mild flu-like illness to fulminant sepsis in a short time period. However, recognition and clinical management of OPSI is not well established. Patients who are asplenic or hyposplenic are at an increased risk for infection and death from encapsulated organisms and other dangerous pathogens. Although relatively rare, it has a high mortality rate with delayed or inadequate treatment, and therefore it is important for Emergency Physicians to be familiar with it. Durations between Splenectomy and onset of OPSI ranged from less than 1 wk to more than 20 years. Although the mortality rate from OPSI has been reduced by appropriate vaccination and education. The precise pathogenesis and a suitable therapeutic strategy remain to be elucidated. Overwhelming postsplenectomy infection (OPSI) is a serious fulminant process that carries a high mortality rate.

OPSI threat in hematological patients.

Overwhelming post-splenectomy infection (OPSI) is a rare medical emergency, mainly caused by encapsulated bacteria, shortly progressing from a mild flu-like syndrome to a fulminant, potentially fatal, sepsis. The risk of OPSI is higher in children and in patients with underlying benign or malignant hematological disorders. We retrospectively assessed OPSI magnitude in a high risk cohort of 162 adult splenectomized patients with malignant (19%) and non malignant (81%) hematological diseases, over a 25-year period: 59 of them splenectomized after immunization against encapsulated bacteria, and 103, splenectomized in the previous 12-year study, receiving only life-long oral penicillin prophylaxis. The influence of splenectomy on the immune system, as well as the incidence, diagnosis, risk factors, preventive measures and management of OPSI are also outlined. OPSI occurred in 7 patients (4%) with a median age of 37 years at time interval from splenectomy ranging from 10 days to 12 years. All OPSIs occurred in non immunized patients, except one fatal Staphylococcus aureus -mediated OPSI in a patient adequately immunized before splenectomy. Our analysis further provides evidence that OPSI is a lifelong risk and that current immune prophylaxis significantly decreases OPSI development. Improvement in patients' education about long-term risk of OPSI and increased physician awareness to face a potentially lethal medical emergency, according to the current surviving sepsis guidelines, represent mandatory strategies for preventing and managing OPSI appropriately.

Cases of Opsi syndrome still candidate for medical ICU

Splenectomized patients are likely to suffer from severe infections, such as sepsis and meningitis, which is called overwhelming postsplenectomy infection (OPSI) syndrome. It seems to be more common in children, but occurs at all ages. The risk is greatest in the early months and years after operation, but never disappears entirely. The course is rapid, the clinical symptoms are serious, and the prognosis is very poor. In this paper, three cases of OPSI syndrome are described, in which infection developed 8, 8 and 15 years after splenectomy; two of the patients died. With the help of these case reports, we want to again emphasize the importance of vaccination, antibiotic prophylaxis and seeking earlier medical attention in splenectomized patients.