Splenectomy For Immune Thrombocytopenia Research Articles

Splenomegaly and Response to Splenectomy in Immune Thrombocytopenia.

Background: Immune thrombocytopenia (ITP) is an acquired autoimmune disorder affecting patients of all ages and backgrounds. While current standards favor medical therapy in the frontline setting, splenectomy remains an integral part of treatment in refractory cases. Ideal parameters for patient selection for surgery remain elusive. Methods: Data for 40 adult patients undergoing splenectomy for ITP at a large urban center between 1 January 2010 and 1 July 2021 were collected and analyzed. Results: Most patients underwent uneventful laparoscopic splenectomy (95%). Complete or partial response at the time of last follow-up occurred in most patients (92.5%), with 60.0% requiring no additional medical therapy following surgery. Thrombosis was the predominant adverse event and the leading cause of death for two patients. Age and presence of splenomegaly appear to be associated with response to splenectomy. Conclusions: Splenectomy remains an effective therapy for selected patients with ITP. Predictors of positive response to splenectomy, such as younger age and the presence of splenomegaly, may help inform clinicians during patient selection for therapy. With strict attention paid to postoperative thromboprophylaxis, the diminishing use of splenectomy may not be warranted.

Proteomics Landscape and Machine Learning Predictor of Response to Splenectomy in Immune Thrombocytopenia

Introduction Immune thrombocytopenia (ITP) is an auto-immune disease characterized by antibody-mediated platelet destruction. Splenectomy remains the effective curative treatment for patients with ITP who failure to respond to TPO-RAs and/or rituximab (Mageau et al, Am J Hematol. 2022). The effectiveness rate of splenectomy for ITP is reported to be roughly 80%, with a 5-year remission rate ranging from 50% to 75% (Chaturvedi et al, Blood. 2018). For subjects presenting with severely low platelet counts or active bleeding, splenectomy represents the primary approach for rapidly increasing platelet levels and potentially saving lives. To achieve personalized and precise treatment, the prediction efficacy on splenectomy remains an urgent problem. Here, we have characterized immune features of ITP patients with splenectomy, and developed a predictive model for assessing the response to splenectomy. Methods Bone marrow plasma samples before splenectomy were collected and analyzed by high-resolution liquid chromatography-tandem mass spectrometry. Weight protein co-expression network analysis (WPCNA) was applied to generate modules from all proteins and mapped them to clinical characteristics. We used least absolute shrinkage and selection operator (Lasso) regression, recursive feature elimination (RFE) for predictors selection. Next, multivariable logistic regression (LR), support vector machine (SVM), and decision tree algorithms were used to develop a predictive model. Results 60 patients with ITP undergoing splenectomy treatment were enrolled into our study, and divided into three groups according to the response [patients with CR (n=30), NR (n=7), and Relapse (n=23)]. A pathway enrichment analysis of WPCNA modules by ClueGO revealed that megakaryocyte differentiation, humoral immune response mediated by circulating immunoglobulin, Fc receptor signaling pathway, and cGMP-PKG signaling pathway were correspondingly enriched in MEtan, MEturquoise, MEblue, and MEpink modules, which were associated with treatments with TPO-RA, IVIG, rhTPO, and post-operation platelet level, respectively. We then performed KEGG pathway enrichment analyses on the different expression proteins (DEPs) to deciphering the immune landscape among each group. For the relapse group, DEPs were primarily enriched in ribosome, spliceosome, neutrophil extracellular trap formation, and necroptosis pathways, comparing with the CR group. For the NR group, most DEPs were enriched in natural killer cell mediated cytotoxicity, NF-kappa B signaling, Fc gamma R-mediated phagocytosis pathways, comparing with the CR group. The models were based on a multi-step predictor pipeline. Inside the pipeline, features were first filtered by lasso regression and RFE algorithm. We identified five proteomic features present in the bone marrow plasm that associated with relapsed to therapy, which included CD40, COL4A3, GALNT6, GSTM3, and UBE2A (Figure). Each ensemble consisted of three algorithms acting in parallel: LR, SVM and decision tree. The AUC values of LR, SVM, and decision tree were 0.962 (95%CI: 0.915-1), 0.957 (95%CI: 0.897-1), and 0.875 (95%CI: 0.782-0.967), respectively. The three algorithms were then tested for performance. The sensitivity, specificity, positive predictive value, and negative predictive value analysis revealed that LR model reached the best performance. Targeting CD40 represents a promising therapeutic approach for enhancing clinical outcomes in patients with ITP who have shown inadequate response following splenectomy. Conclusion Our analysis revealed that ITP patients with splenectomy showed distinct immune characteristics when achieving different response. Protein features extracted from bone marrow plasma can be selected as predictors of response to splenectomy.

Improving the chances of response to splenectomy in immune thrombocytopenia.

Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by isolated thrombocytopenia. Its pathogenesis is complex relying in large part on destruction of platelets recognized by autoantibodies within the spleen. However, other mechanisms, such as platelet desialylation, may play a role in platelet reduction by accelerating their clearance in the liver. In their study, Mendoza and colleagues reported on platelet scintigraphy performed in 51 ITP patients, showing a response in 87.5% when the sequestration occurred in the spleen versus 45% in case of non-splenic destruction. Platelet desialylation was also measured after splenectomy and found to be higher in non-responder patients. These latter results, while requiring confirmation prior to splenectomy, support platelet desialylation may also be a potential biomarker of non-response to splenectomy. Commentary on: Mendoza etal. Study of platelet kinetics in immune thrombocytopenia to predict splenectomy response. Br J Haematol 2024;204:315-323.

Indium-labelled autologous platelet sequestration studies predict response to splenectomy in immune thrombocytopenia: an Australian experience.

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

Splenectomy Results in Venous Thromboembolic Events in Women: A Nurses Health Study

Background:Splenectomy is a therapy for many conditions the most common of which are trauma, Hodgkin lymphoma, thalassemia and hereditary or autoimmune hemolytic anemia, and immune thrombocytopenia (ITP). By 1960 splenectomy had been known to create a risk of over-whelming post splenectomy sepsis (OPSS) with a high mortality rate. More recently population-based studies from Denmark have suggested that there is also an increased risk of thromboembolic (TEE) complications. Thromboembolic events generally include venous thrombosis (deep vein thrombosis (DVT) and pulmonary embolism (PE)) and arterial thrombosis (stroke and myocardial infarction [MI]). ITP and hemolytic anemias have been shown to independently have increased risks of arterial and venous TEE which has complicated assessment of additive effects of splenectomy. While splenectomized patients appear to be at increased risk of arterial and venous thromboses, the incidence and which TEEs predominate remains unclear as does the timing of occurrence of the TEEs.Method:The Nurses' Health Study (NHS) was established in 1976 and enrolled 121,700 female nurses between 30-55 years old. The nurses completed baseline questionnaires and biennial follow up questionnaires. In 2004, NHS participants were asked whether they had undergone splenectomy. The primary outcomes of this study included DVT,PE, and MI, which were identified through self-report on biennial questionnaires. MIs were confirmed through medical record review. End of follow-up for this study went through 2016, date of death, or diagnosis of outcome, whichever came first. Loss to follow up was very low and causes of deaths were carefully tracked.Descriptive statistics compared the splenectomy vs non-splenectomy participants with respect to basic demographic factors and variables that may be related to the outcomes. Cox proportional hazards models were run to evaluate incidence rates for primary and secondary outcomes. We conducted age-adjusted, as well as multivariable adjusted models. Multivariate models were adjusted for age, body mass index, smoking history, diabetes mellitus, high blood pressure, and high cholesterol, thus taking into account known predictors of the specific TEEs. Multivariate models were updated every two years to account for time varying confounders.Results：In 2004, 323 participants reported having had a splenectomy, out of 96,000 completing the questionnaire. There was a strong, significant association between splenectomy and subsequent DVT (n=613) and PE (n=840). The multivariate adjusted HR was 3.73 (95%CI: 1.77-7.86) and 3.80 (95%CI: 2.04-7.10) respectively. When considered together as a composite outcome, the splenectomized participants had a 3.52-fold higher risk (95%CI: 2.12-5.85) of PE/DVT compared with non-splenectomy. There was no association between splenectomy and MI (n=1011; HR = 0.97, 95% CI: 0.31-3.01). Given the very limited number of stroke cases in the splenectomized population, we were underpowered/unable to evaluate the association of splenectomy and subsequent risk of stroke.Conclusion:This study found that splenectomized patients are at a 4-fold increased risk of PE and DVT as compared to not-splenectomized individuals. These findings were not dependent on any of the potentially confounding variable analyzed. We found no association between splenectomy and MI. There are limitations to these results. No information was available on family history and perioperative thrombo-prophylaxis. Also we do not know exactly when or why splenectomy was performed; there were at least 34 women who had splenectomy for ITP but in most cases the underlying disease was unknown. Since the nurses in 1976 were already between 30-55 years old, it is no longer possible to retrospectively obtain sufficient information. Therefore, the splenectomy question has been added to the current survey for NHS#2. We will pursue the relevant information in a follow up letter to respondents who reported having undergone splenectomy while seeing if the findings of NHS#2 confirm those of NHS#1. [Display omitted] DisclosuresBussel: Principia/Sanofi: Consultancy, Membership on an entity's Board of Directors or advisory committees; UCB: Consultancy, Membership on an entity's Board of Directors or advisory committees, Other: DSMB; Momenta/Janssen: Consultancy, Membership on an entity's Board of Directors or advisory committees; Argenx: Consultancy, Membership on an entity's Board of Directors or advisory committees; Dova/Sobi: Consultancy, Membership on an entity's Board of Directors or advisory committees; CSL: Other: DSMB; RallyBio: Consultancy, Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees; Amgen: Consultancy, Membership on an entity's Board of Directors or advisory committees; Rigel: Consultancy, Membership on an entity's Board of Directors or advisory committees; UptoDate: Honoraria.

Splenectomy in İmmune Thrombocytopenia: A Retrospective Analysis of 25-Year Follow-up Data from a Tertiary Health Clinic.

Immune thrombocytopenia (ITP) is a rare autoimmune disorder presenting with isolated thrombocytopenia. Splenectomy is still one of the treatment alternatives for these patients. Here we aim to analyze long term follow-up data of splenectomy in immune thrombocytopenia. This retrospectively designed study was conducted in a tertiary health clinic. Patients with ITP who were splenectomized between 1990 and 2015 were included. Response to treatment was interpreted as 'complete response', 'response' or 'no response'. The incidence of response loss was evaluated.Perioperative and long term complications and overall survival rates were determined.Out of 51 patients, who underwent splenectomy after 12 months of diagnosis, 47 achieved a response (92.2%). Of 47 patients who had a platelet count at least 30.000/µL, 41 (87.2%) had CR. Incidence of loss of response was 10.5% (95% confidence interval (CI): 4%-26.1%) at 30 months.Two patients died, and overall survival rate was 97.4% (95% CI: 82.8%-99.6%) at 30 months of follow up. Considering the complications: two patients had venous thromboembolism, 11 had minor bleeding episodes and 15 suffered from perioperative infections. Our study suggests that splenectomy promises a high level of response with acceptable complication rates. Although less preferred recently, splenectomy should still be taken into consideration when remission is not achieved especially after 12 months of disease.

Effectiveness and Reliability of Splenectomy in Chronic Immune Thrombocytopenia

Although splenectomy is markedly effective treatment modality at early period in adult chronic immune thrombocytopenia (ITP), long-term outcomes are still controversial. The aim of this study was to determine long-term effectiveness and reliability of splenectomy in ITP and to identify factors influencing on long-term response. The study included 100 ITP patients who underwent splenectomy in our General Surgery Department between 1980 and 2004. Parameters including gender, age, platelet count, comorbid diseases, antiplatelet antibody positivity and long-term effect of medical treatment on response to splenectomy were considered. Owing to advances in treatment protocols, ITP has become a more benign disease and need for splenectomy has been decreased. However, splenectomy is a highly effective in second-line treatment. Based on our results, it was seen that open or laparoscopic splenectomy with low morbidity and mortality rates is an appropriate treatment modality for long-term control of chronic ITP in cases requiring splenectomy. Age and postoperative platelet count were identified as significant prognostic and predictive factors for long-term response to splenectomy.

Autologous platelet scintigraphy and clinical outcome of splenectomy in immune thrombocytopenia: A systematic review and meta-analysis.

Autologous platelet sequestration pattern is associated with post-splenectomy platelet response in patients with immune thrombocytopenia (ITP). However, published results are contradictory, and have not been systematically reviewed. Our aim is to systematically review and meta-analyse the association between sequestration pattern and post-splenectomy platelet response. Articles were selected from MEDLINE when they a) included ITP patients, b) performed scintigraphy, and c) included post-splenectomy platelet response. The 23 included studies (published between 1969-2018) represented 2966 ITP-patients. Response to splenectomy occurred most frequently in patients with a splenic pattern (87.1 % in splenic versus 47.1 % in mixed and 25.5 % in hepatic patterns). A pooled analysis of 8 studies showed an odds ratio of 14.21 (95 % CI: 3.65-55.37) for platelet response in the splenic versus the hepatic group. Our findings indicate that a splenic sequestration pattern is associated with better response after splenectomy. Platelet sequestration patterns may be useful in the clinical decision-making regarding splenectomy.

Absolute lymphocyte count is a predictor of outcome after splenectomy for immune thrombocytopenia

Absolute lymphocyte count is a predictor of outcome after splenectomy for immune thrombocytopenia

AB006. Robotic splenectomy for im mune thrombocytopenia: should this be routine practice?

Background: Immune thrombocytopenia (ITP) is an acquired thrombocytopenia due to autoantibody-mediated destruction of platelets. Splenectomy is reserved for patients with thrombocytopenia that is refractory to medical therapies, and those with significant bleeding issues. The objective of this case report is to highlight the benefit of the robotic platform in performing minimally invasive splenectomy.

Trends of Splenectomy in Pediatric Patients with Immune Thrombocytopenia before and after the ASH 2011 Evidence-Based Practice Guidelines

Background: Immune thrombocytopenia (ITP) is an autoimmune disorder which affects 1 in 10,000 children. Thrombocytopenia occurs largely due to rapid destruction of antibody sensitized platelets in the reticuloendothelial system. While spontaneous resolution is commonly seen in acute ITP, treatment of chronic or persistent ITP is controversial and could pose as management challenges for clinicians. Splenectomy increases platelet survival by removing a common site of platelet destruction and has a 70-80% response rate, however, published literature is conflicting regarding its ideal timing. Furthermore, it is an invasive procedure with significant risk of infections, venous thromboembolism. In 2011, the American Society of Hematology (ASH) published revised clinical practice guidelines for the management of ITP (Neunert C et al. Blood 2011). Splenectomy was recommended in patients with severe symptomatic ITP with significant or persistent bleeding refractory to intravenous immunoglobulin (IVIG), corticosteroids, and anti-D therapy. It is unclear if the rates of splenectomy have changed since the publication of these guidelines. Using a large population-based estimate, we aimed to study the trends of splenectomy in pediatric patients with ITP, and the factors predicting the procedure in this population.Methods: We used discharge data from the National (Nationwide) Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality (AHRQ). Pediatric patients (age ≤ 18 years) hospitalized with ITP from 2005-2014 were identified using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD9-CM) code 287.31. Cases of total splenectomy were identified using ICD9-CM procedure code ‘41.50‘. To calculate estimated trends of splenectomy, Cochran-Armitage trend test was used. Chi-square test, Mann-Whitney U test, survey logistic regression and hierarchal regression were used for analyses. Weights provided by NIS were used to calculate national estimates. A p-value < 0.05 was considered significant. All analyses were performed using SAS 9.4 (SAS Institute Inc., Cary, NC, USA).Results: The NIS data included 62 million weighted pediatric hospitalizations between 2005 and 2014 (unweighted admissions n=13 million). After excluding patient transfers, a total of 40,998 weighted hospitalizations with ITP occurred during this time-period. Total splenectomy was performed in 1,009 patients. Splenectomy rate showed a decline prior to revised guidelines: 3.3% (2005-06) to 2.3% (2011-12). It continued to decline further after the publication: 1.6% (2013-14), p <0.0001. Similar trend was noted for patients < 5 years of age: 10.6% (2005-06), 5.2% (2011-12), and 3.5% (2013-14), p <0.001. None of the other patient ages (5-10, 11-15, and 16-18), gender, or hospital regions showed consistent splenectomy trend in either direction. Multivariable analysis showed one year increase in patient age significantly increased adjusted odds of having splenectomy (odds ratio, OR 1.18, 95% confidence interval, CI 1.14-1.22, p <0.0001). Having an intracranial bleed also significantly predicted splenectomy (OR 5.16, 95% CI 1.96- 13.61, p=0.0009). Splenectomy was more commonly done in patients from Midwest (OR 2.83, 95% CI 1.19-6.74, p= 0.02) and South region (OR 1.89, 95% CI 1.02-3.51, p=0.04) compared to Northeast region. Mortality rate was similar between patients who received splenectomy and who did not.Conclusion: Overall, splenectomy rates showed consistently downward trend in pediatric patients with ITP between 2005 and 2014. Reduction in splenectomy rates in children <5 years of age could be due to increasing awareness for sepsis secondary to asplenia. Presence of severe bleeding (e.g. intracranial bleeding) in patients with ITP predicted splenectomy, which is consistent with ASH recommendations. Declining splenectomy rates in last 3 years since ASH guidelines release could mean that more patients receive medical management initially as recommended by ASH. However, due to possible under-coding for IVIG, corticosteroids, and anti-D and lack of longitudinal data, we were unable to study the association between prior treatment and splenectomy. Future studies should revisit the role of splenectomy in ITP with advent of novel therapeutic options such as thrombopoietin receptor agonists. DisclosuresNo relevant conflicts of interest to declare.

Splenectomy for immune thrombocytopenia in systemic lupus erythromatosus in Nigeria

Systemic lupus erythromatosus (SLE), is an autoimmune disease characterized by autoreactive T cells and polyclonal activation of B cells. SLE is a rare disease in tropical Africa and its association with thrombocytopenia has not been reported in the English literature. This is a case of a 37-year-old female civil servant with SLE complicated by refractory thrombocytopenia and successfully managed with splenectomy. Splenectomy in the management of thrombocytopenia associated with SLE in Africans as in other regions of the world is indicated in cases refractory to other treatment options.

The spleen dictates platelet destruction, anti-platelet antibody production, and lymphocyte distribution patterns in a murine model of immune thrombocytopenia.

For many years, splenectomy has been used to treat immune thrombocytopenia (ITP), and this procedure benefits approximately two-thirds of the treated patients. Although splenectomy may raise platelet counts, antibody-coated platelets and cytotoxic T lymphocytes appear to persist or can change over time. To better understand how the spleen may affect anti-platelet immune responses, we used a murine model of ITP demonstrating both antibody-mediated and T lymphocyte-mediated thrombocytopenia. Mice with severe combined immunodeficiency (SCID) were either splenectomized or not and transfused with splenocytes from CD61 (GPIIIa) knockout mice immunized against CD61(+) platelets. Platelet counts and anti-platelet antibody levels were performed weekly. After 4weeks, the mice were sacrificed, and lymphoid organs were harvested and examined by flow cytometry to quantify CD4(+)CD25(+)FoxP3(+) Tregs and conventional cross-presenting XCR1(+) and tolerizing SIRPα+ dendritic cells. The results indicate that compared with control non-splenectomized mice, thrombocytopenia was improved and anti-platelet antibody production was significantly diminished in all splenectomized mice that received immune splenocytes. Splenectomized SCID mice also had a marked reduction in Tregs in the thymus together with an increased proportion of both thymic dendritic cell subsets that correlated with increased platelet counts. Of interest, although splenectomy diminished anti-platelet antibody production and raised platelet counts, marrow megakaryocyte densities were still significantly reduced in mice that received immune splenocytes. These results suggest that the spleen in murine ITP not only is the primary site responsible for platelet destruction, but it also controls, to a significant extent, the antibody response against platelets and the migration patterns of lymphocyte subsets.

The role of laparoscopy in the identification and management of missing accessory spleens after primary splenectomy: A case report and literature review

We present a 7-year-old boy with recurrent thrombocytopenia after primary laparoscopic splenectomy for immune thrombocytopenia (ITP). Imaging modalities (ultrasound, computed tomography scan, and scintigraphy) revealed two accessory spleens while the subsequent second laparoscopy revealed 11, which were successfully removed. The relevant medical literature is reviewed, and the value of laparoscopy for chronic ITP is highlighted.

The Effect Of Insurance Status and Comorbidities On Outcomes After Splenectomy Among Patients With ITP

BackgroundImportance of individual's insurance status in access and quality of health care has been well documented in published literature. Lack of or inadequate insurance may be associated with negative health consequences. Currently there are no studies that have examined the relation between the insurance status and splenectomy outcomes among patients with Immune thrombocytopenia (ITP)Splenectomy has been the standard second-line treatment for adults with ITP for several decades. Recently published guidelines by American Society of Hematology for the management of ITP gave splenectomy a strong recommendation based on its sustained remission rates and long-term experience. Despite its effectiveness in achieving durable response, there has been a tendency among physicians to avoid or delay splenectomy due to the fear of removing a healthy organ, a long-term risk of sepsis and mortality associated with surgery.Previously reported mortality rates for splenectomy in ITP were around 0.2 - 1%. However, the effect of insurance status and the prevalence of common comorbidities that can influence postoperative outcomes have not been explored in this population. The following study, representative of a national cohort of ITP patients who underwent splenectomy, assesses the effect of insurance type and comorbidities on postoperative mortality and length of stay in hospital following splenectomy.We hypothesized that uninsured or federally funded health insurance and underlying comorbidities might have an unfavorable effect on the outcomes. MethodsWithin the Health Care Utilization Project Nationwide Inpatient Sample (NIS), we identified 9419 (n=9419) patients who underwent splenectomy for ITP between 2004-2009. The presence of comorbidities was assessed based on Elixhauser's comorbid conditions and patients were categorized based on presence of ≥ 3, ≥2 or ≤1 comorbid conditions. Multiple logistic regression analyses was performed to evaluate the effect of insurance type on inpatient mortality rate after adjusting for age, sex, race, and number of comorbidities. Furthermore, the effect of comorbidities on inpatient mortality was analyzed after adjusting for other variables such as age, type of insurance and race. Hospital length of stay (LOS) was dichotomized as prolonged if LOS > 9 days (third quartile) or short if LOS< 9 days. The study protocol was approved by Case Western Reserve University Institutional Review Board. ResultsDuring the six-year period between 2004-2009, a weighted estimate of 9419 patients were identified who underwent splenectomy for ITP. Inpatient postoperative mortality was 3.1% and overall median LOS was 9 days. Most patients were privately insured (47.6%), followed by Medicare (35.1%), Medicaid (8.6%) and uninsured (8.5%). 2% of Medicare, 0.2% of Medicaid, 0.7% of uninsured and 0.16% of privately insured patients died. On multivariable analysis there was no significant difference in mortality among Medicare, Medicaid and uninsured patients when compared to privately insured patients. Medicaid patients had prolonged length of stay as compared to privately insured patients (aOR 2.55, CI 1.71-3.79, P< 0.0001). Patients with higher comorbidities had higher mortality rates and length of stay after adjusting for age, race and insurance type. Patients with ≥ 3 comorbid conditions were three times more likely to die (aOR 3.03, CI 1.4-6.2, p<0.01) and six times more likely to have prolonged hospital stay (aOR 6.4, CI 4.8-8.6, p<0.001) as compared to patients with ≤1 comorbid condition. ConclusionPatients who are privately insured have similar mortality rates as compared to Medicare and Medicaid and uninsured population. Preoperative comorbidities are associated with increased risk of mortality, and risk adjustment is necessary while evaluating patients with ITP undergoing splenectomy. Disclosures:No relevant conflicts of interest to declare.

Complications of Splenectomy for Immune Thrombocytopenia

The management of immune thrombocytopenia (ITP) is continuing to evolve with the introduction of new agents such as rituximab and thrombopoietic drugs

Late but effective long-term response to splenectomy in the treatment of immune thrombocytopenia

Late but effective long-term response to splenectomy in the treatment of immune thrombocytopenia

Vascular Endothelial Cell Function in Catastrophic Antiphospholipid Syndrome: A Case Report and Review of the Literature

Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune condition, which has been associated with a high mortality rate. However, with current management that includes a combination of anticoagulation, glucocorticoid administration, and plasma exchange, mortality rate has declined. Despite survival improvement with new generation immunosuppressive agents, their mechanisms of action are poorly defined, and CAPS is still considered a high-risk complication in patients known with antiphospholipid antibody syndrome. Herein, we present a case of a 79-year-old male who presented with a myocardial infarct and renal failure secondary to CAPS following a splenectomy for immune thrombocytopenia. Regardless of rapid combination of first-line treatment and rituximab therapy, the patient developed lethal cardiogenic shock secondary to mitral valve papillary muscle necrosis. Discussion of the pathophysiology and avenues of future therapies in CAPS are reported.

Proteomics-based identification of haptoglobin as a favourable serum biomarker for predicting long-term response to splenectomy in patients with primary immune thrombocytopenia

BackgroundSplenectomy is the most effective treatment for patients with primary immune thrombocytopenia (ITP) who fail to respond to steroid therapy. Thus far, there is no effective means to predict the long-term haematological response of the procedure. The purpose of this study was to identify serum biomarkers as predictors of long-term response based on a proteomics approach.MethodsThe serum samples of ITP patients were collected before splenectomy and seven days after surgery. After depletion of the abundant serum proteins, pooled preoperative serum samples from four responders to splenectomy, four nonresponders and four healthy controls were subjected to two-dimensional gel electrophoresis (2-DE). Nine protein spots with at least a five-fold alteration in expression between responders and nonresponders were all identified as haptoglobin (Hp) by matrix-assisted laser desorption/ionisation time-of-flight (MALDI-TOF) mass spectrometer (MS) analysis. The validation of serum Hp expression was performed using enzyme-linked immunosorbent assays (ELISA) in thirty-seven responders, thirteen nonresponders and twenty-one healthy controls.ResultsThe preoperative serum levels of Hp in the nonresponders (925.9 ± 293.5 μg/ml) were significantly lower than those in the responders (1417.4 ± 315.0 μg/ml, p <0.001) and the healthy controls (1409.1 ± 354.2 μg/ml, p <0.001), while there was no significant difference between the latter two groups. The postoperative serum levels of Hp in responders and nonresponders were (1414.1 ± 225.0 μg/ml) and (952.9 ± 202.4 μg/ml), respectively. There were no significant differences between the serum Hp levels before and after surgery in both responders and nonresponders (p>0.05). The preoperative serum levels of Hp did not significantly correlate with preoperative platelet count of the same blood samples (r = 0.244, p = 0.087), while it positively correlated with postoperative peak platelet count (r = 0.622, p < 0.001). The optimal cutoff value of preoperative serum Hp levels (1173.80 μg/ml) derived from the receiver operating characteristic (ROC) curve led to 78.4% sensitivity and 84.6% specificity.ConclusionsThese results suggest that serum Hp levels may serve as a favourable predictor for the long-term response to splenectomy in ITP and may help to understand the pathophysiological differences between responders and nonresponders.

Durability and factors associated with long term response after splenectomy for primary immune thrombocytopenia (ITP) and outcome of relapsed or refractory patients

Splenectomy is the usual form of therapy for immune thrombocytopena (ITP) after steroid failure. We retrospectively studied the data in adult patients who underwent splenectomy for ITP from July 1996 to June 2008 to evaluate the long term responses, clinical and laboratory factors associated with long term responses and outcome of relapsed or refractory patients. Thirty eight patients, 30 (79%) females, with a median age of 23 years (range 15–69), underwent splenectomy. The procedure was laparoscopic in 28 (73.5%) and open in 10 patients. Splenectomy resulted in a response in 34/38 (89.5%) patients and failed in four (10.5%) patients. After a median follow-up of 58 months (range 7–144), 24 (63%) patients had a maintained response without treatment (platelet count of >50 × 109/l). Most of the relapses occurred during the first year but two patients had late relapses. There were procedure-related complications in seven (18.0%) patients but no cases of overwhelming sepsis. Only four relapsed or refractory patients had a platelet count below 50 × 109/l at the last follow-up indicating response to alternative therapies. Responsiveness to steroids before the procedure (p = 0.025) along with a platelet count of ≥150 × 109/l at 4 weeks (p = <0.0001) and a highest platelet count of ≥400 × 109/l at any time post-splenectomy (p = 0.005), were associated with a long term response in univariate analysis. In conclusion, splenectomy remains an effective treatment for ITP after steroid failure in terms of long term responses, and the majority of relapsed or refractory patients respond to alternative therapies.