Fatal overwhelming postsplenectomy infection due to Streptococcus pneumoniae serotype 10A with atypical polysaccharide capsule in a patient with chromosome 22q11.2 deletion syndrome: A case report.

We report the first case of a teenage patient with chromosome 22q11.2 deletion syndrome who died of overwhelming postsplenectomy infection (OPSI) by Streptococcus pneumoniae despite appropriate prevention by pneumococcal vaccine. He had congenital heart disease and underwent several surgeries. Immunodeficiency had not been noticed clinically. Two years prior to death, splenectomy was performed for a drug-resistant idiopathic thrombocytopenic purpura and he was immunized with 23-valent pneumococcal polysaccharide vaccine (PPV23) 4 months after splenectomy. He died suddenly after a mild flu-like symptom. Autopsy was performed and OPSI was diagnosed. Blood culture was positive for S. pneumoniae. This isolated S. pneumoniae strain was serotypically un-typable by polyvalent serum agglutination test. On the contrary, multilocus sequence typing followed by DNA sequencing indicated the molecular serotype as 10A. Additional testing using monovalent and factor-specific sera confirmed the strain as serotype 10A. Ultrastructural observation of this S. pneumoniae strain showed that the polysaccharide capsule was thin and sparse. We speculate that the abnormal morphology of the capsule may have accounted for the polyvalent serum agglutination failure and may possibly be associated with severity of OPSI observed in this case. Chromosome 22q11.2 deletion syndrome is associated with certain immunodeficiency, especially susceptible to S. pneumoniae infections; however, fatal OPSI has not been reported. In addition to vaccination, prophylactic antibiotics may be necessary for these patients who are at risk of immunodeficiency.

Overwhelming Postsplenectomy Infection: A Prospective Multicenter Cohort Study.

BACKGROUND: Recent population-based cohort studies have questioned the role of pneumococci as the most frequent pathogen causing severe infection in patients after splenectomy. The aim of the study was to define the causative pathogens and clinical presentation of patients with overwhelming postsplenectomy infection (OPSI). METHODS: In a prospective cohort study in 173 German intensive care units, we searched for patients with and without asplenia and community-acquired severe sepsis/septic shock. Clinical and laboratory variables and survival of patients were assessed. RESULTS: Fifty-two patients with severe sepsis or septic shock with asplenia and 52 without asplenia were included. OPSI patients more often had a history of malignancy (38% vs 17%; P = .016) and had a lower body mass index (24 kg/m(2) vs 28 kg/m(2); P = .004). Streptococcus pneumoniae was detected more frequently in OPSI patients (42% vs 12% without asplenia; P < .001) and more frequently manifested as bloodstream infection (31% vs 6%; P = .002). Gram-negative infection was similar in both groups (12% vs 19%; P = .157). Pneumococcal vaccine coverage of OPSI patients was low overall (42% vs 8% among patients without asplenia; P < .001). Purpura fulminans was a frequent complication, developing in 19% of OPSI patients vs 5% of patients without asplenia (P = .038). The interval between splenectomy and OPSI was 6 years (range, 1 month-50 years). On multivariable Poisson regression, asplenia was the only predictive variable independently associated with pneumococcal sepsis (adjusted relative risk, 2.53 [95% confidence interval, 1.06-6.08]). CONCLUSIONS: Pneumococcal infections remain the most important cause of severe sepsis and septic shock following splenectomy.

Intravenous Immunoglobulin for Overwhelming Postsplenectomy Infection.

Overwhelming postsplenectomy infection (OPSI) is a life-threatening condition causing fulminant bacteremia in asplenic patients. Intravenous immunoglobulin (IVIG) therapy is theoretically effective for OPSI. Herein, we present a case of OPSI treated successfully with IVIG, along with results of a literature review. An asplenic 70-year-old male with acute ischemic stroke presented with rapid and fulminant septic shock from pneumococcus pneumonia and bacteremia. Resuscitation and antibiotics including IVIG therapy were instituted. The patient survived with favorable outcomes. We analyzed all case reports or case series of OPSI from 1971 through 2017. Cases with IVIG treatment showed a significantly higher survival rate than those without IVIG, even with multivariable regression analysis, suggesting IVIG as an independent predictive factor for survival. It suggests that IVIG is effective for OPSI and that it can be regarded as an adjunctive treatment option for OPSI.

Asplenia and Hyposplenism: An Underrecognized Immune Deficiency.

The number of disorders associated with congenital or acquired asplenia and functional hyposplenism has increased substantially over the past couple decades. Previously, screening for asplenia and hyposplenism was a barrier to identifying patients at risk. Recent methods for measuring splenic function have emerged as accurate and reliable. Identifying patients prevents overwhelming postsplenectomy infection or invasive pneumococcal disease. Approaches to protect patients with asplenia or hyposplenism include patient education of risks and signs/symptoms of infection, vaccination, and antibiotic prophylaxis. Physicians have evaluated methods of preserving splenic function after trauma and sought alternative treatments of refractory cytopenias treated with splenectomy in the past.

A case report of bacteremia manifesting as an overwhelming postsplenectomy infection due to Streptococcus pneumoniae post vaccination.

A 62-year-old woman was admitted for acute epigastralgia and high-grade fever of over 39 °C. The patient had undergone splenectomy for idiopathic portal hypertension 1 year ago and vaccination against Streptococcus pneumoniae immediately post operation. She developed localized peritoneal irritation and abdominal distension. Her serum creatinine had increased to 1.5 mg/dL and procalcitonin was 12.5 ng/ml. Computed tomography of the abdomen revealed edematous large intestine and increased ascites. From these results, the patient was considered to have spontaneous bacterial peritonitis (SBP). Vancomycin (VCM) and doripenem (DRPM) were administered to control the infection. Unexpectedly, S. pneumoniae was detected in the blood culture. Hence, ampicillin/sulbactam was administered after discontinuing VCM. The patient recovered without any life-threatening complications and was discharged after 10 days. In conclusion, overwhelming postsplenectomy infection (OPSI) due to S. pneumoniae could develop in patient with splenectomy even after vaccination. Although the bacteremia probably due to SBP and acute renal dysfunction was accompanied by OPSI, our patient recovered rapidly.

Effects of a pharmacy-driven perisplenectomy vaccination program on vaccination rates and adherence to guidelines.

BACKGROUND: Overwhelming postsplenectomy infection is a serious potential outcome for patients who have undergone resection of the spleen and is associated with a high mortality rate. The most common bacterial causes are the encapsulated organisms Streptococcus pneumoniae, Neisseria meningitidis, and Hemophilus influenzae type B, all of which are vaccine-preventable. Current guidelines recommend vaccination against these 3 bacteria, but adherence to these guidelines is less than ideal. In 2007, a "perisplenectomy vaccination kit" was introduced at the authors' institution to improve compliance with immunization guidelines by making the vaccines and necessary information for patients and providers more readily available. OBJECTIVE: To evaluate and compare vaccination rates for patients who underwent splenectomy before and after introduction of the perisplenectomy vaccination kit and, secondarily, to identify any characteristics unique to those who did not receive appropriate perisplenectomy vaccinations. METHODS: In this observational study, performed at the QEII Health Sciences Centre of Capital Health in Halifax, Nova Scotia, data were reviewed for patients who underwent splenectomy between 2008 and 2011. Vaccination rates and other descriptive statistics were calculated and compared with data for a 3-year period before implementation of the program. RESULTS: Vaccination rates in the 3-year period following implementation of the perisplenectomy vaccination kit were 100% against S. pneumoniae, 97% against N. meningitidis, and 93% against H. influenzae type B. The corresponding rates in the 3 years before introduction of the kit were 91%, 75%, and 68%, respectively. No characteristics predicting inadequate immunization were identified in univariate or multivariate analysis. CONCLUSION: Introduction of a pharmacy-driven perisplenectomy vaccination kit program improved rates of appropriate vaccination for patients who underwent splenectomy.

CONTEXTE: L'infection fulminante post-splénectomie, une complication potentielle sérieuse chez les patients ayant subi une ablation de la rate, présente un taux de mortalité élevé. Les causes bactériennes les plus fréquentes de cette infection sont les bactéries encapsulées Streptococcus pneumoniae, Neisseria meningitidis et Haemophilus influenzae de type b, qui peuvent toutes être prévenues par un vaccin. Les lignes directrices actuelles recommandent une vaccination contre ces trois espèces de bactéries, mais le respect de ces lignes directrices est loin d'être idéal. En 2007, une « trousse de vaccination périsplénectomie ¼ a été adoptée dans l'établissement des auteurs. Cette trousse avait pour but d'améliorer le degré de conformité aux lignes directrices sur l'immunisation en facilitant l'accès aux vaccins et aux renseignements nécessaires pour les patients et les professionnels de la santé. OBJECTIF: Déterminer quels étaient les taux de vaccination des patients ayant subi une splénectomie avant et après la mise en place de la « trousse de vaccination périsplénectomie ¼, puis comparer ces valeurs; et identifier toute caractéristique propre à ceux qui n'ont pas reçu les vaccins nécessaires durant la période entourant la splénectomie. MÉTHODES: Dans cette étude observationnelle menée au QEII Health Sciences Centre de Capital Health à Halifax (Nouvelle-Écosse), on a procédé à l'analyse des données concernant les patients ayant subi une splénectomie entre 2008 et 2011. Les taux de vaccination ainsi que d'autres statistiques descriptives ont été calculés et comparés à des données d'une période de trois ans avant la mise en œuvre du programme d'utilisation de la « trousse de vaccination périsplénectomie ¼. RÉSULTATS: Les taux de vaccination de la période de trois ans qui a suivi la mise en œuvre de la « trousse de vaccination périsplénectomie ¼ étaient de 100 % pour S. pneumoniae, 97 % pour N. meningitidis et 93 % pour H. influenzae de type b. Les taux correspondants des trois années précédant cette mise en œuvre étaient respectivement de 91 %, 75 % et 68 %. Aucun prédicteur d'une immunisation insuffisante n'a été cerné par l'analyse univariée ou multivariée. CONCLUSION: L'implantation d'un programme de « trousse de vaccination périsplénectomie ¼ mené par les pharmaciens a permis d'accroître les taux d'administration des vaccins qui sont indiqués chez les patients ayant subi une splénectomie. [Traduction par l'éditeur].

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.

A Case of Pneumococcal Endocarditis Complicating Overwhelming Postsplenectomy Infection / 日本心臓血管外科学会雑誌

Postsplenectomy patients are susceptible to severe infections due to encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae and this condition is widely known as Overwhelming Postsplenectomy Infection (OPSI). OPSI is relatively rare in conditions with rapid progression and high mortality rates. There are very few reports of infective endocarditis from OPSI. A 40-year-old man who underwent splenectomy 20 years before was transported to the emergency room. He had severe heart failure and multiple cerebral embolisms. The echocardiography showed vegetation on the aortic valve and severe regurgitation due to perforation of the non-coronary cusp. We performed aortic valve replacement on an emergent basis. In infected patients after splenectomy, early diagnosis and prompt treatment are desired because of the possibility of rapid progression of sepsis in encapsulated bacteria and poor prognosis.

Síndrome de sepsis grave postesplenectomía secundario a meningococcemia / Overwhelming postsplenectomy infection (OPSI) syndrome secondary to meningococcemia

Resumen Los pacientes esplenectomizados o con hipoesplenia son susceptibles a desarrollar infecciones y sepsis grave. A esta entidad se le denomina sídrome de sepsis postesplenectomía (SSPE). Reportamos el caso de una paciente que presentó SSPE secundario a Neisseria meningitidis serogrupo CR7, 10 años después de practicársele esplenectomía por púrpura trombocitopénica idiopática. El SSPE tiene una elevada mortalidad, su evolución es fulminante, las manifestaciones son graves y el pronóstico es malo. Es fundamental que los pacientes esplenectomizados sean vacunados, reciban antibióticos profilácticos y atención médica ante cualquier manifestación de un proceso infeccioso. Es prioritario implementar una política de salud pública para el desarrollo de guías relacionadas al seguimiento de los pacientes esplenectomizados y sobre la profilaxis, diagnóstico y manejo del SSPE, y difundir entre los pacientes esplenectomizados y con disfunción esplénica así como al grupo médico tratante programas educacionales relacionados con esta enfermedad.

Abstract Patients with splenectomy or splenic dysfunction are likely to suffer from severe infections and sepsis. This syndrome is called overwhelming postsplenectomy infection (OPSI). We present the case of an adult who developed OPSI syndrome secondary to Neisseria meningitidis serogroup CR 7, ten years after being splenectomized due to idiopathic thrombocytopenic purpura. OPSI syndrome has a high mortality. Its course is fulminating, the clinical symptoms are serious and the prognosis is poor. It is important for splenectomized patients to receive immunizations, antibiotic prophylaxis and seek medical attention at the earliest sign of minor infection. Public health politics should be implemented for the development of guidelines related to the monitoring of splenecto-mized patients and prophylaxis, diagnosis and treatment of OPSI syndrome and make reliable information on this disease available to patients and physicians.

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.