Overwhelming Postsplenectomy Infections Research Articles

Survival Secrets: Unmasking the Factors Predicting Failure of Non-operative Management (NOM) in Splenic Injuries.

Splenic injuries are common solid organ injuries resulting from blunt abdominal trauma in road traffic accidents. Very often, splenic injuries can be life-threatening. Earlier, splenic injuries were often dealt with surgical intervention, such as splenectomy. With the recognition of the immunological function of the spleen and possible complications of splenectomy surgery, such as overwhelming post-splenectomy infections (OPSI), there has been a recent trend for non-operative management (NOM). To study the variables predicting failure of NOM in blunt abdominal trauma patients with splenic injury. This is a retrospective study that includes 235 patients who presented to the Safdarjung Hospital emergency room (New Delhi, India) with blunt trauma abdomen and splenic injuries with or without associated injuries between January 2019 and December 2021. The data was entered in a Microsoft Excel spreadsheet (Microsoft Corp., Redmond, WA, USA). Categorical variables were expressed as frequencies and percentages. Pearson's chi-square test of association was used to determine if there is a relationship between two variables. A p-value of <0.05 was considered statistically significant. Out of 235 patients with blunt abdominal trauma and splenic injuries, 82 were hemodynamically unstable despite resuscitation and were taken up for emergency laparotomy. The remaining 153 patients, who were either hemodynamically stable or stabilized after adequate resuscitation, were managed on the lines of NOM. The number of patients with splenic injury in AAST grades 1, 2, 3, 4, and 5 was 36, 50, 40, 24, and three, respectively. Out of 153 patients, 130 (85%) were successfully managed by NOM, while eight (5%) had to discontinue NOM as they required surgical intervention. The failure of NOM (fNOM) is seen mostly with grade 5 injuries (2/2, 100%, p<0.01), followed by grade 4 (4/20, 20%) and grade 3 (2/37, 5.7%). The mean age in fNOM was 58.3 years, as compared to 42.2 years in the success of NOM (sNOM). All eight patients had multiple concomitant injuries, with femur fracture being the most common association in up to six patients (p<0.01), followed by liver injury in four patients. There were 15 mortalities, irrespective of AAST severity grade. All of these patients had associated concomitant injuries, with intracranial bleeding (n = 10, 32%, p<0.01) being the most common association, followed by femur fracture (n = 6, 20%) and liver injury (n = 5, 16%). Also, the cause of death was unrelated to splenic trauma (p = 0.67), with pulmonary embolism (n = 6, 40%, p<0.01) being the most common cause, followed by brain stem herniation (n = 5, 34%). Non-operative management is a safe and efficient method for treating patients with splenic injuries who are hemodynamically stable or stabilized. The factors associated with fNOM include elderly age, a higher American Association for the Surgery of Trauma(AAST) grade of splenic injury, and associated concomitant injuries. Femur fracture was the most common concomitant injury present in cases where NOM failed, followed by liver injury. The presence of intracranial bleeds in these patients was a common association with mortality, irrespective of the grade of splenic injury.

Asplenia and spleen hypofunction.

Asplenia (the congenital or acquired absence of the spleen) and hyposplenism (defective spleen function) are common causes of morbidity and mortality. The spleen is a secondary lymphoid organ that is responsible for the regulation of immune responses and blood filtration. Hence, asplenia or hyposplenism increases susceptibility to severe and invasive infections, especially those sustained by encapsulated bacteria (namely, Neisseria meningitidis, Streptococcus pneumoniae, Haemophilus influenzae type b). Asplenia is predominantly due to splenectomy for either traumatic events or oncohaematological conditions. Hyposplenism can be caused by several conditions, including haematological, infectious, autoimmune and gastrointestinal disorders. Anatomical disruption of the spleen and depletion of immune cells, especially IgM memory B cells, seem to be predominantly responsible for the clinical manifestations. Early recognition of hyposplenism and proper management of asplenia are warranted to prevent overwhelming post-splenectomy infections through vaccination and antibiotic prophylaxis. Although recommendations are available, the implementation of vaccination strategies, including more effective and immunogenic vaccines, is needed. Additionally, screening programmes for early detection of hyposplenism in high-risk patients and improvement of patient education are warranted.

Congenital asplenia study: clinical and laboratory characterisation of adults with congenital asplenia.

Congenital asplenia is a rare disorder commonly associated with other visceral and cardiac congenital anomalies. Isolated congenital asplenia is even less common than syndromic forms. The risk of severe bacterial infections associated with asplenia is the most concerning clinical implication and carries a significant mortality risk. Prophylactic measures against the clinical syndrome known as overwhelming postsplenectomy infections (OPSI) include vaccination, prophylactic and emergency antibiotics and health education including fever management and travel advice. This case series describes fourteen adults with congenital asplenia and polysplenia syndrome, most of whom were diagnosed incidentally as adults, and outlines the nature of their diagnosis, clinical phenotype, family history and key pathology findings.

Factors Influencing Pneumococcal Vaccination-Induced Total IgG, IgG2, and IgA Levels in Vaccinated Splenectomized Patients

Splenectomy is closely associated with a lifetime risk of pneumococcal and other encapsulated bacterial infections. In this study, it was aimed to investigate the change of antibody levels after vaccination against Streptococcus pneumoniae according to age, gender, years after splenectomy and the possible effect of splenectomy on IgG avidity. In addition the education and awareness levels of the participants about post-splenectomy vaccination and infectious diseases were also analyzed. In the first of the three phases of this study, 32 individuals with splenectomy were enrolled. The awareness of the patients about the possible risks after splenectomy was investigated with a simple questionnaire. Routine laboratory test results were obtained and clinical examinations were performed. In the second stage, total Ig values of 29 splenectomy patients were determined. In the third phase, 14 splenectomy and 5 healthy volunteers were vaccinated according to the Vaccination Practices Advisory Committee (ACIP) guidelines. Pneumococcal-specific antibody levels and IgG avidity were detected by enzyme linked immunosorbent assay (ELISA). It was determined that 68.8% of the splenectomized patients were unaware of their vaccination status and 78.2% of them were unaware of the increased risk of infectious diseases in asplenic conditions. . According to the hospital information management system, all 31 (96.87%) patients, except one, were vaccinated with PPV23. As expected, vaccinated patients exhibited high levels of vaccine-specific antibody production with IgG, IgG2, and IgA antibody concentrations of 321 ± 76.68 mg/l, 73.07 ± 8.273 mg/l, and 117.8 ± 14.94 mg/l, respectively, but unvaccinated patients had very low antibody (IgG, IgG2 and IgA antibody concentrations were 11.5 mg/l, 1.3 mg/l and 1.2 mg/l, respectively) levels. Although there was no correlation between antibody titers and gender, age groups or presence of fever history, the decrease in total IgG, IgG2 and IgA titers were strongly correlated with the time since splenectomy. Antibody titers were found to be significantly lower in splenectomized patients vaccinated more than 10 years ago. Routine laboratory results were at normal levels except for low platelet count. On the other hand, both splenectomized and healthy control subjects displayed similar IgG avidity index values (%61.8 ve %64.4% inhibition in control and splenectomized subjects, respectively) after the vaccination schedule. It was shown that post-splenectomy vaccination with PPV23 induced high levels of pneumococcus-specific antibody production that can last for more than five years. It was determined that more efforts should be made to increase the level of knowledge about pneumococcal and other overwhelming post-splenectomy infections (OPSI) as the awareness of the patients about the risks of infection after splenectomy was poor. In particular, patients with splenectomy operation more than 10 years ago should be very careful about being asplenic as they were determined to have significantly lower level of vaccine-specific antibody production. Our study was also the first to show that splenectomy does not alter IgG avidity induced by pneumococcal vaccination.

Considerations in laparoscopic resection of giant pancreatic cystic neoplasms

Laparoscopic distal pancreatectomy (LDP) with (LDPS) or without splenectomy for cystic tumours in the body and tail has become the standard of care. Data on patients with large tumours of the body and tail of the pancreas are sparse. A retrospective analysis of a prospectively maintained database of patients who were managed with laparoscopic surgery for pancreatic cystic neoplasm since 2010 was done. Patients with cysts more than 8 cm were analysed. Clinical presentation, imaging, details of the surgical procedure and the outcomes were looked into. Five patients of giant pancreatic cystic neoplasm (GPCN) were managed with LDPS. Four patients were female, mean age was 45 years (range 15-69 years). The mean cyst size was 11.2 cms (range 8-15 cm). The splenic vein was either stretched or thrombosed in all patients. Three patients had sinistral portal hypertension. All patients were operated with a modified five-port placement. None of the patients required conversion. Mean operative duration was 3½ h, blood loss was 80 ml approximately and none required a blood transfusion. One patient had a biochemical leak. All patients were discharged from the hospital by 3rd postoperative day. Drain removal was done before discharge except in the patient with biochemical leak (removed on day 6). On a median follow-up of 89 months (range 1-120 months), two patients developed diabetes. There has been no Overwhelming post-splenectomy infections (OPSI). Laparoscopic distal pancreatectomy is feasible in patients with GPCN and offers the all the short-term benefits, namely lesser pain, no wound infections, early return of bowel activity, early return to orals and early discharge and early return to work. Splenectomy was required in all patients because of splenic vein thrombosis and portal hypertension in three and for technical reasons in the rest.

Disseminated lymph node tuberculosis after splenectomy: an unusual case report in an adolescent

BackgroundSplenectomized patients are at an increased risk for overwhelming post-splenectomy infections typically with encapsulated bacteria. The clinical association between splenectomy and lymph-node tuberculosis is unclear.Case presentationWe describe a rare case of disseminated tuberculous lymphadenitis in an 18-year-old woman with history of splenectomy because of hereditary sherocytosis. She was admitted with enlargement of bilateral-cervical and left-axillary lymph nodes and fever. A diagnosis of probable tuberculosis was made based on the findings of fine-needle aspiration. Histology showed granulomas and extensive caseous necrosis, with the site of puncture located at an enlarged lymph node on the right side. The diagnosis was confirmed via nucleic-acid amplification tests following excisional biopsy of the left axillary lymph node. Disseminated tuberculous lymphadenitis was localized in the bilateral neck, right lung hilum, left sub-axillary region, and mediastinum, as detected from contrast-enhanced computed tomography of the neck.ConclusionsMycobacterium tuberculosis infection should be considered in children and adolescents with extensive enlargement of lymph nodes after splenectomy.

Splenektomi Yapılan Hastalarda Aşılanma Oranları ve Kılavuzlara Uyum: Gözlemsel, Kesitsel Bir Çalışma

Introduction: Splenectomy, whatever the reason, is an absolute indication for vaccination against encapsulated bacteria in order to prevent overwhelming post-splenectomy infections. This study aimed to determine compliance to immunization guidelines for splenectomy in General Surgery Clinics. Materials and Methods: In this observational cross-sectional study, we included patients who underwent splenectomy between April 2016 and April 2018, and recorded the patient data and vaccination status. Results: Splenectomy was performed in 65 patients, 40 (61.5%) were male and median age was 56 (19-90) years. Six of the operations were emergent, 21 were planned and 38 were urgent mostly due to solid organ malignancy. Only 14 (21.5%) patients were vaccinated with Haemophilus influenzae, meningococcal and pneumococcal vaccines according to the guidelines (≥2 weeks before the operation). 14 of the 21 patients (66.6%) who underwent planned splenectomy were vaccinated appropriately. Conclusion: Compliance with vaccination guidelines was low before splenectomy operations. There is a room for improvement particularly for patients undergoing elective splenectomy. A patient-centered team strategy is required to vaccinate patients at the stage of splenectomy planning.

Individualized concept for the treatment of autosomal recessive polycystic kidney disease with end-stage renal disease.

Management of children with autosomal recessive polycystic kidney disease (ARPKD) who develop end-stage renal disease (ESRD) remains challenging because of concomitant liver disease. Patients with recurrent cholangitis are candidates for liver-kidney transplantation, while the treatment for patients with splenomegaly and pancytopenia due to portal hypertension is controversial. Herein, we report 7 children who were treated using an individualized treatment strategy stratified by liver disease. Two patients with recurrent cholangitis underwent sequential liver-kidney transplantation, while 4 patients with splenomegaly and pancytopenia but without recurrent cholangitis underwent splenectomy followed by isolated kidney transplantation. The remaining patient, who did not have cholangitis and pancytopenia, underwent isolated kidney transplantation. Blood cell counts were normalized after splenectomy was performed at the median age of 8.7 (range, 7.4-11.7) years. Kidney transplantation was performed at the median age of 8.8 (range, 1.9-14.7) years in all patients. Overwhelming post-splenectomy infections and cholangitis did not occur during the median follow-up period of 6.3 (range, 1.0-13.2) years. The estimated glomerular filtration rate at the last follow-up was 53 (range, 35-107) mL/min/1.73m2 . No graft loss occurred. Our individualized treatment strategy stratified by recurrent cholangitis and pancytopenia can be a feasible strategy for children with ARPKD who develop ESRD and warrants further evaluation.

Pneumococcal purpura fulminans in asplenic or hyposplenic patients: a French multicenter exposed-unexposed retrospective cohort study

BackgroundPneumococcal infections remain the main cause of overwhelming post-splenectomy infections, and purpura fulminans may develop in almost 20% of patients with overwhelming post-splenectomy infection. We aimed at describing the impact of asplenia/hyposplenia on the clinical features and the outcomes of adult patients admitted to the intensive care unit (ICU) for pneumococcal purpura fulminans.MethodsA 17-year national multicenter retrospective cohort study included adult patients admitted to 55 French ICUs for an infectious purpura fulminans from 2000 to 2016. Patients with pneumococcal purpura fulminans were analyzed according to the absence or presence of asplenia/hyposplenia.ResultsAmong the 306 patients admitted to the ICU for purpura fulminans, 67 (22%) had a pneumococcal purpura fulminans, of whom 34 (51%) had asplenia (n = 29/34, 85%) or hyposplenia (n = 5/34, 15%) and 33 (49%) had eusplenia. The prevalence of pneumococcal purpura fulminans was seven times higher in asplenic/hyposplenic patients compared to eusplenic patients with purpura fulminans (n = 34/39, 87% vs. n = 33/267, 12%; p < 0.001). The median time interval between the occurrence of asplenia/hyposplenia and ICU admission was 20 [9–32] years. Pneumococcal vaccine coverage was 35% in asplenic/hyposplenic patients. Purpura was more frequently reported before ICU admission in asplenic/hyposplenic patients (n = 25/34, 73% vs. n = 13/33, 39%; p = 0.01). The rate of bacteremia did not differ between asplenic/hyposplenic and eusplenic patients (n = 31/34, 91% vs n = 27/33, 82%; p = 0.261). SAPS II (60 ± 14 vs. 60 ± 18; p = 0.244) and SOFA (13 [1–5] vs. 14 [1–4, 6]; p = 0.48) scores did not differ between asplenic/hyposplenic and eusplenic patients. There were no significant differences between asplenic/hyposplenic and eusplenic patients regarding the rate of limb amputation (n = 9/34, 26% vs. 15/33, 45%; p = 0.11) and hospital mortality (n = 20/34, 59% vs. n = 15/33, 45%; p = 0.27).ConclusionsHalf of pneumococcal purpura fulminans episodes occurred in asplenic or hyposplenic patients. Pneumococcal vaccine coverage was reported in one third of asplenic/hyposplenic patients. Half of pneumococcal purpura fulminans episodes occurred more than 20 years after splenectomy. Outcomes of pneumococcal purpura fulminans did not show significant differences between patients with or without asplenia or hyposplenia, although the small number of patients included limited our power to detect potential differences between groups.

Splenic autotransplantation: a systematic review.

Splenectomy is a surgical procedure indicated in a variety of medical conditions including trauma. Post-operatively, there is a lifelong risk of developing overwhelming sepsis from encapsulated bacteria, most commonly due to Streptococcus pneumoniae. Splenic autotransplantation has been proposed as a method to recover splenic function in patients requiring splenectomy with otherwise normal spleens. This study aims to systematically review the literature to determine the efficacy of spleen autotransplantation. MEDLINE, PubMed and the Cochrane Library were searched for all studies assessing splenic autotransplantation (January 1947 to July 2018). Data were extracted on study characteristics, outcomes assessed, including spleen scintigraphy results, blood film counts and serum immunoglobulin (Ig) levels. Data were obtained from 18 primary studies. All studies demonstrated return of regenerated spleen tissue in the majority of their patients (95.3%) on spleen scintigraphy. In 12 studies, 90.2% of patients had blood films return to normal following transplantation. Ig levels were shown to return to normal in all 12 studies where it was assessed. In 11 studies, 3.7% of patients had post-operative complications. In five studies, 1.3% of patients had post-operative infections in the follow-up period. Splenic autotransplantation is a safe procedure with minimal complications that can return splenic filtration function and Ig levels to normal ranges. It has not been confirmed whether autotransplantation provides meaningful protection against overwhelming post-splenectomy infections.

Splenectomy in the Surgery Department “A” at the University Hospital Point G Bamako

We conducted a retrospective study that included patients with erythrocyte and hemolytic pathology justifying a splenectomy and patients presenting splenic trauma for which hemostasis splenectomy is required. We performed 27 total splenectomy in which 26 are by laparotomy (92.86%) and 1 by laparoscopy (3.57%). A partial splenectomy was performed by laparoscopy. The morbidity was marked by 2 cases of infectious syndrome and 1 case severe anemia. The mortality was 7.14% (n = 2). Splenectomy is part of the therapeutic arsenal for benign or malignant hematological disorders that constitute the main indication for elective splenectomy. Splenectomy was one of the most common operations in abdominal surgery. During the past decade, an increased rate of late complications, specially septic and thromboembolic complications are well documented. The risk is related to the indication of splenectomy, and is less than 1% in adults without immunodeficiency. However, these overwhelming postsplenectomy infections are associated with a high mortality rate. The best treatment of these infections is preventive measures which are based on vaccination and education of asplenic patients.

Anti-Pneumococcal Vaccine-Induced Cellular Immune Responses in Post-Traumatic Splenectomized Individuals.

Splenectomy is associated with increased risk of overwhelming post-splenectomy infections despite proper anti-pneumococcal vaccination. As most studies concentrated on vaccination-induced humoral immunity, the cellular immune responses triggered in splenectomized patients are not yet well studied. The present study aims to investigate this area as it can contribute to the development of more effective vaccination strategies. Five healthy and 14 splenectomized patients were vaccinated with pneumococcal conjugate polysaccharide vaccine (PCV) followed by pneumococcal polysaccharide vaccine according to the guidelines established by Advisory Committee on Immunization Practices. PBMC samples collected 0, 8, and 12weeks after PCV immunization were in vitro stimulated with PCV. Levels of lymphoproliferation, TH cell differentiation, and cytokine release were assessed by carboxyfluorescein succinimidyl ester labeling, intracellular cytokine staining, and ELISA, respectively. While TH1-dominated immune response was detected in both groups, asplenic individuals generated significantly lower levels of TH1 cells following in vitro stimulation. Similarly, levels of IFN-γ, IL-4, and IL-17 release and lymphoproliferation were significantly lower in asplenic patients. According to our data, splenectomy negatively influences the levels of PCV-induced lymphoproliferation, TH1 differentiation, and cytokine release. Besides, PCV failed to induce TH17-dominant immune response which is crucial for protection against extracellular pathogens.

Outcome of Partial Splenectomy for Hypersplenism in Children with Thalassemia

Background: Total splenectomy, exposes children to the high risk of overwhelming postsplenectomy infections (OPSI). To avoid these adverse consequences, partial splenectomy has long been practiced for thalasseemia in children. It has been reported that the partial splenectomy keeps the child immunologically competent, hematologically stable with minimum blood transfusion and makes their life more comfortable in comparison to total splenectomy.Objectives: To compare the results of partial and total splenectomy.Methodology: This prospective interventional comperative study was done in the department of Pediatric Surgery, BSMMU from 2010 to 2012. Children who underwent partial splenectomy were considered as the case and who underwent total splenectomy as the control. Number of blood (RCC) transfusions (ml/ kg/year), Peripheral blood film (Hb%, WBC count, platelet count, Howell-Jolly body, serum bilirubin), volume of liver (ml), volume of spleen (ml), number of OPSI case, were compared between the case and control groups both pre and postoperatively.Results: Postsplenectomy blood transfusion requirement is comparatively more decreased in control group than case group. The inter group difference at 6 month is significant (p= 0.004). Peripheral blood pictures are improved in both groups. Post splenectomy hemoglobin level was increased in both groups but it was maintained at a more static fashion in control group than case group (P = 0.114). Howell-jolly body in the partial splenectomy group disappeared almost completely at month 6, while the same inclusion body in the total splenectomy group appeared in all the children (p= 0.001). There was no postsplenectomy infection in case group while two found in control group. After partial splenectomy the residual volume of the spleen was gradually increasing. The increase in volume of the liver was notably greater in the total splenectomy group than that in the partial splenectomy group (p&lt; 0.05).Conclusion: Partial splenectomy in patients with ²-thalassemia is effective in controlling hemolysis, improving peripheral blood picture while preserving the residual splenic phagocytic and immune function.J. Paediatr. Surg. Bangladesh 6(2): 39-46, 2015 (July)

Vaccinations in asplenic adults.

The recommended immunizations for adult asplenic patients are reviewed. Patients without a spleen are at risk of developing overwhelming postsplenectomy infections due to encapsulated organisms, mainly pneumococcal, meningococcal, and Haemophilus influenzae type b (Hib). Due to the high mortality rates associated with these infections, vaccinations are recommended as a preventive measure. It is challenging to ensure optimal immunizations in these high-risk patients due to the number of recommended vaccines, the availability of multiple formulations, and the inability to administer specific formulations at the same time, as well as differences in subsequent vaccine administration schedules. Pharmacists play a key role in recommending specific vaccines and timing for these patients in order to achieve the most robust immune response. This article reviews the specific recommendations for pneumococcal, meningococcal, Hib, and influenza vaccinations in asplenic patients. In order to prevent potentially life-threatening infections, asplenic individuals should be vaccinated against S. pneumoniae, N. meningitidis, Hib, and influenza. The optimal timing of vaccination in relation to splenectomy depends on the nature of the splenectomy.

Giant unilocular hydatid cyst of spleen

Background: Hydatid cyst of spleen is a very rare condition. It is usually secondary to spread of cysts from other regions. Primary hydatid cyst of spleen without involvement of any other organ is even rarer. Here, we report a case of a giant unilocular hydatid cyst of spleen without involvement of any other organ. The patient underwent laparotomy and splenectomy was done. This case report aims at reporting a rare case of a primary giant unilocular hydatid cyst of spleen and describes its management. Case Presentation: A 45 year old female presented to the surgical outpatient department with complaint of fever, dull dragging ache and lump in the left upper abdomen. On examination there was a slightly tender lump in the left hypochondrium suggestive of splenomegaly. Ultrasonography and computed tomography of abdomen were suggestive of a giant unilocular splenic hydatid cyst. The patient was operated and splenectomy was performed. The patient was discharged on albendazole therapy and the follow-up was uneventful. Histopathological examination of the specimen confirmed the presence of hydatid cyst. Conclusion: Primary hydatid cyst of spleen is a very rare condition. Surgery remains the mainstay of treatment. The evolving concept is towards splenic preservation surgery with cyst enucleation to prevent overwhelming post-splenectomy infections.

Treatment Of Infection-Associated Purpura Fulminans With Protein C Zymogen Is Associated With a High Survival Rate

Purpura fulminans (PF) is rapidly progressing, life-threatening disorder, characterized by skin lesions with a typical morphology, disseminated intravascular coagulopathy, multiple organ failure, septic shock, most often, but not exclusively caused by infections (meningococci, pneumococci, and others). It is associated with a breakdown of the protein C system, an important regulator of blood coagulation, leading to consumption coagulopathy, intravascular fibrin deposition, downregulated fibrinolysis, disturbance of microcirculation and finally death from multiple organ failure. Mortality of severe sepsis with coagulopathy is as high as 80-100%, and persistent disabilities (i.e. amputations) are frequent in survivors. Several case series including more than 340 patients, suggest that substitution of protein C zymogen can impressively improve coagulopathy, reduce amputation rate and improve survival compared to historical controls in PF associated with sepsis in neonates, children and adults, encouraging some centers to incorporate it in their local guidelines for treatment of purpura fulminans.We report a series of 9 consecutive patients with purpura fulminans, treated with a plasma-derived protein C zymogen concentrate (Ceprotin®, Baxter, Vienna, Austria) for acute onset PF in 3 Vienna hospitals, in which the treatment guidelines included protein C replacement. Median age was 29 years (range 2 months to 73 years), 5 male, 4 female, 5 adult, 4 pediatric patients were treated, respectively. Six patients had meningococcal sepsis, 2 had overwhelming post-splenectomy infections, and 1 had heat-shock induced coagulopathy. All patients presented with typical skin lesions and acute critical illness and were admitted to intensive care units. Coagulopathy was present in 100%, severe vasopressor-dependent sepsis in 100%, acute renal failure in 89%, respiratory failure in 89%. Median SAPS II score in the adults was 78 (range 45-97), predicting a mortality of 78.3% (range 31-88.5%). All pediatric patients had a Glasgow Meningococcal Septicemia prognostic score >8, indicating a fatal outcome. Renal replacement therapy was necessary in 56%, mechanical ventilation in 89%. Initial protein C levels were markedly decreased in the 8 patients with infections.Standardized sepsis therapy was applied according to the surviving sepsis guidelines. Protein C was given as an initial bolus infusion (100 U/kg) followed by a continuous infusion with 10 U/kg/h, adjusted to obtain plasma protein C activity levels of 1.0 U/mL. In addition, platelet, red blood cell, fibrinogen and antithrombin concentrates were given as needed.In one patient (PF caused by heat shock) protein C infusion was stopped after 2 days, and he died after 10 days from refractory multiple organ failure. All other patients (with infection-induced PF) survived. Coagulopathy resolved within a few days, and all patients could successfully be weaned from intensive care therapy. Organ function was completely restored without residual dysfunction. One patient needed amputation of both forefeet and nose reconstruction, 6 patients had, in part extended, scar formation at the skin. Median follow up was 8 months (range 2-20). At that time all patients were fully active without apparent limitations.In conclusion, standardized, full-code sepsis therapy, together with protein C substitution, resulted in very high survival rate of patients with infection-induced PF (as compared to predicted mortality) and a low rate of disabilities in this otherwise deleterious disease. As Ceprotin® is approved only for congenital protein C deficiency, controlled clinical studies are urgently needed to gain more scientific evidence for this potentially life-saving, but still off-label therapy in patients with PF. Disclosures:Knoebl:Novo Nordisk: Consultancy, Honoraria; Baxter: Consultancy, Honoraria. Off Label Use: plasma derived protein C concentrate (Ceprotin(R)) for acquired protein C deficiency in purpura fulminans.

Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients

Splenectomy has been used in patients with common variable immunodeficiency disorders (CVID), mainly in the context of refractory autoimmune cytopenia and suspected lymphoma, but there are understandable concerns about the potential of compounding an existing immunodeficiency. With increasing use of rituximab as an alternative treatment for refractory autoimmune cytopenia, the role of splenectomy in CVID needs to be re-examined. This retrospective study provides the largest cohesive data set to date describing the outcome of splenectomy in 45 CVID patients in the past 40 years. Splenectomy proved to be an effective long-term treatment in 75% of CVID patients with autoimmune cytopenia, even in some cases when rituximab had failed. Splenectomy does not worsen mortality in CVID and adequate immunoglobulin replacement therapy appears to play a protective role in overwhelming post-splenectomy infections. Future trials comparing the effectiveness and safety of rituximab and splenectomy are needed to provide clearer guidance on the second-line management of autoimmune cytopenia in CVID.

Der splenektomierte Patient in der Praxis

Asplenia is found in a very heterogeneous group of patients with a wide range of age and comorbidities. These patients have an increased risk of overwhelming post-splenectomy infections (OPSI) even several decades after splenectomy. The mortality rate associated with OPSI is 30 - 60 %, which is why the immunisation against encapsulated bacteria is mandatory. In addition these patients show an increased risk of thrombosis of the portal venous system. In the present article all the necessary, during daily practice often neglected preventive measures and behavioural rules are discussed.

Fatal overwhelming postsplenectomy infection caused by Streptococcus pneumoniae in mothers within 1 year after delivery: case report

Invasive pneumococcal disease (IPD) has a high mortality. Although preventive strategies including vaccination have been established for children, less attention has been devoted to pregnant and postnatal women and to mothers caring for their infants. As a significant number of women have either undergone splenectomy or are in a hyposplenic state and have not received adequate pneumococcal vaccinations, they can potentially develop overwhelming postsplenectomy infections. A 34-year-old woman with an 8-month-old baby, who underwent splenectomy at the age of 10 for benign pancreatic tumor, presented with fever and petechial eruption. Despite extensive treatment, she died 17h after admission. A 40-year-old woman with a 11-month-old baby, who underwent splenectomy at 2years of age for hemolytic anemia, was admitted for septic shock and disseminated intravascular coagulation. Despite extensive treatment, she died 2h after admission. Blood cultures from both women were positive for Streptococcus pneumoniae and neither of them had been vaccinated against the bacterium. IPD rapidly progressed and developed to multiple organ dysfunction syndromes in mothers caring for their infants, particularly those who had undergone splenectomy or were in a hyposplenic state. Thus, routine pneumococcal vaccination is recommended for pregnant women. In addition, we suggest a thorough medical interview and checkup for splenectomy or hyposplenism in prenatal women.

Excellent long-term outcomes of ABO-incompatible kidney transplantation in children

Due to profound shortage of suitable deceased allografts, much effort has been made to investigate whether successful kidney transplantation is possible across the ABO blood group barrier even for pediatric recipients. Methods Twenty-five pediatric patients underwent ABO-incompatible living kidney transplantation. All patients received three sessions of plasmapheresis (PP) or immunoadsorption (IA) to remove the anti-A/B antibodies before transplantation. This was followed by splenectomy and a conventional quadruple drug immunosuppressive protocol. To prevent overwhelming postsplenectomy infections, penicillin prophylaxis as well as pretransplant pneumococcal vaccine was given to all patients. Results Of 25 patients, 12 grafts had rebound increases in their anti-A/B isoagglutinin titers within 10 days posttransplant. Moreover, 10 of 12 (83%) patients developed acute rejection with the increase of isoagglutinins. One patient lost the graft because of uncontrolled delayed hyperacute rejection, while the other nine acute rejection episodes were reversed with a standard anti-rejection therapy in combination with PP/IA. Four patients had severe bacterial infectious complications after transplantation. The actuarial 1-, 5- and 10-year graft survival rates are 96%, 91% and 91%, respectively. Conclusions With the adequate desensitization protocol, ABO-incompatible kidney transplantation is a valid alternative even for children with end stage renal disease.