Splenectomized Patients Research Articles

Splenectomy Reports

Introduction It was seen that splenectomy creates a disability situation in an individual, and in order to eliminate it, people applied to health boards to get a report to eliminate their social and economic losses recognized to them. Objective To examine the reason for surgery, method of surgery and the type of report they wanted to receive in splenectomised patients who applied to the general surgery committee polyclinic in 2017-2018-2019-2020 and 2023 when the pandemic ended. Materials and Methods Patients who applied to general surgery outpatient clinics were asked whether they had any surgery related to general surgery, and epicrises and pathology results of splenectomised patients were seen and recorded. Results Of the 23 splenectomised patients, 15 were female and 8 were male. Of the 15 female patients, 3 were splenectomised for ovarian ca, 3 for gastric ca, 2 for distal pancreatic ca, 2 for lymphoma, 1 for colon ca, 1 for traumatic cause, 2 for ITP, 1 for sarcoidosis. In male patients, 4 were splenectomised for traumatic, 1 for colon ca, 1 for ITP, 1 for thalassemia major and 1 for CML. The mean age of female patients was 48.1 years and the mean age of male patients was 37.4 years. The most common reason for splenectomy in women was malignancy and the most common reason for splenectomy in men was trauma. Conclusion Splenectomized patients had applied to receive the most DSR.

Erythema Migrans in Patients with Post-Traumatic Splenectomy.

Information on asplenic Lyme borreliosis (LB) patients with erythema migrans (EM) is lacking. We compared the course and outcome of 26 EM episodes in 24 post-trauma splenectomized patients (median age 51 years) diagnosed at a single clinical center in Slovenia during 1994-2023 with those of 52 age- and sex-matched patients with EM but with no history of splenectomy. All patients were followed for one year. A comparison of pre-treatment characteristics revealed that EM in splenectomized patients was of shorter duration before diagnosis (4 vs. 8 days, p = 0.034) with a smaller EM diameter (10.5 vs. 14 cm, p = 0.046), and more frequently fulfilled criteria for disseminated LB (3/26, 11.5% vs. 0%, p = 0.034). Treatment failure occurred in 5/26 (19.2%) EM episodes in splenectomized patients versus 0/52 in non-splenectomized patients (p = 0.003). The five treatment failure cases were retreated with antibiotic regimens used to treat EM and had complete resolution of all symptoms/signs. In conclusion, our study showed that splenectomized adult patients with EM differ somewhat in presentation and more often have treatment failure compared with non-splenectomized patients with EM.

236 Post-Splenectomy Patients: Reaudit of Current Practice at Living Well Partnership PCN

Abstract Aim Post-splenectomy patients are at increased risk of sepsis and overwhelming post-splenectomy infection (OPSI). This reaudit aimed to look at a primary care network’s (PCN) compliance with established immunisation guidelines and antibiotic prophylaxis post-splenectomy. Method A retrospective study of 29 splenectomy or asplenia patients between 2018-2022 was conducted within a primary care network. Hospital and GP records were reviewed for antibiotic prescriptions (prophylactic and emergency), vaccination records, electronic splenectomy alert and admission for infection. Interventions included a 15-minute patient intervention GP consultation and correction of EMIS coding. Results The 2018 audit showed that a proportion of splenectomy patients were not appropriately prescribed antibiotics. There was lack of patient insight into their condition (asplenia v splenectomy). An active, patient-centred intervention was employed to improve rates of antibiotic prescribing, perform counselling on regular recommended vaccinations and ensure correct clinical coding for follow up. Compliance to audit criteria increased across all measures between the 3 PDSA cycles. Antibiotic prophylaxis prescription increased from 53% (2018) to 71% in 2022. Emergency antibiotic prescription increased from 6% (2018) to 39% in 2022. Influenza vaccination rates increased from 71% (2018) to 82% in 2022. Following the intervention consultation, 85% of patients had appropriate antibiotic prophylaxis prescription and 64% had appropriate emergency antibiotics prescribed. Conclusions Prescription for emergency and prophylactic antibiotics, and correct coding for annual and 5-year vaccinations were improved for this cohort of splenectomy and asplenia patients. Patients valued a structured consultation leading to improved understanding and self-management.

A retrospective survey on follow-up of splenectomy patients due to β-thalassemia and Sickle cell Anemia in Karbala, Iraq during 2010-2023

Background: Hemoglobinopathy is considered a common monogenetic genetic disorder worldwide. Splenectomy is considered a therapeutic strategy in patients with hemoglobinopathy. The aim of current study was to provide a survey on the splenectomy and 5 years follow-up in different clinical forms of β-thalassemia (intermedia, Major) and Sickle cell Anemia (SCA) patients who referred to Hereditary Blood Disease Center in the Karbala Teaching Hospital for Children in Karbala, Iraq. Materials and Methods: In this retrospective study we tried to evaluate 126 hemoglobinopathy and thalassemia patients from Karbala City, Iraq. All cases of splenectomy due to hemoglobinopathy and thalassemia during 2010-2023 who referred to the Hereditary Blood Disease Center in the Karbala Teaching Hospital for Children in Karbala, Iraq were included. Patient data was collected at three-time points. The first was after the splenectomy, the second during 1-5 years, and the third step after 5 years. Clinical and laboratory data were retrieved from the patient’s file. Results: The mean age of splenectomy of included patients was 14.1±7.5 years. From 126 cases, 103 (81.74%) were β-Thalassemia, 13 (10.32%) were SCA, and 10 (7.94%) were Sickle cell beta-thalassemia. The mean age in SCA was significantly less than two other groups (mean age in β-Thalassemia, SCA and Sickle cell beta thalassemia were 18.2±8.7, 24.2±12.7 and 25.2±9.5, respectively) (p=0.008). Platelet and WBC count represents a significant increase during 1-5 years after splenectomy in comparison with 1 year after splenectomy (for Platelet and WBC p=0.03 and 0.001, respectively). Conclusion: splenectomy is considered the last therapeutic option in hemoglobinopathy patients. All Hemoglobinopathy patients represented significant improvement after splenectomy. Because there was no suitable treatment in the past, splenectomy was considered a therapeutic solution. It should be said that periodic follow-up of splenectomy patients in hemoglobinopathy plays an important role in improving the management of these diseases.

Splenectomy as a part of cytoreductive surgery in ovarian cancer: systematic review and meta-analysis

ObjectiveThe role of splenectomy on cytoreductive surgery in patients with ovarian cancer remains controversial. We conducted this meta-analysis to evaluate the safety and impact of survival outcome of splenectomy in...

Protective effects of COVID-19 vaccination in splenectomized patients with immune thrombocytopenia.

Splenectomy is an effective treatment for immune thrombocytopenia (ITP). The effect of COVID-19 vaccination on splenectomized patients with ITP during the COVID-19 pandemic has not been reported. Therefore, this study aimed to investigate the effect of COVID-19 vaccination on clinical outcomes in these patients. This was a longitudinal study of splenectomized patients with ITP. A total of 191 splenectomized patients were included in this study. After a median follow-up of 114 months, 146 (76.4%) patients had a sustained response to splenectomy. During COVID-19 infection, vaccinated patients showed a lower risk of severe infections (odds ratio [OR], 0.13; 95% confidence interval [CI]: 0.05-0.36; p < 0.001), hospitalization (OR, 0.13; 95% CI, 0.04-0.48; p = 0.002), and ITP exacerbation (OR, 0.16; 95% CI, 0.04-0.67; p = 0.012). These findings indicate that COVID-19 vaccination plays a protective role in splenectomized patients with ITP.

Splenectomy as Part of Maximal-Effort Cytoreductive Surgery in Advanced Epithelial Ovarian Cancer.

A splenectomy is frequently performed during debulking surgery for advanced ovarian cancer. Its impact on perioperative and survival outcomes remains questionable as current evidence is conflicting. In the present study, we sought to determine the factors that affect survival rates in ovarian cancer patients that undergo a splenectomy as part of maximal-effort cytoreduction. A retrospective chart review was conducted that included all epithelial ovarian cancer patients that had surgical cytoreduction for advanced epithelial ovarian cancer. Differences among splenectomized and non splenectomized patients were evaluated as well as the impact of known risk factors on survival outcomes of splenectomized patients. Overall, 245 patients were identified and 223 were included in the present series, of whom 91 had a splenectomy. Recurrence rates as well as death rates were comparable among splenectomized and non-splenectomized patients; however, both the disease-free survival (log-rank = 0.001), as well as the overall survival of splenectomized patients (log-rank = 0.006), was shorter. Thrombotic events as well as rates of pulmonary embolism were comparable. Sepsis was more common among splenectomized patients. The site of splenic metastases did not influence patients' survival. Among splenectomized patients, those offered primary debulking had longer progression-free survival (log-rank = 0.042), although their overall survival did not differ compared to patients submitted to interval debulking. Complete debulking significantly improved the overall survival compared to optimal debulking (log-rank = 0.047). Splenectomized patients that developed sepsis had worse overall survival (log-rank = 0.005). The findings of our study support the feasibility of splenectomy in advanced epithelial ovarian cancer; however, its impact on patients' survival is considerable. Therefore, every effort should be made to avoid splenic injury which will result in unintended splenectomy for non-oncological reasons.

EGS P13 Adherence to Guidelines for Post-Splenectomy Patients in a Primary Care Practice in North West England

Abstract Background Splenectomy is indicated in haematological, oncological disorders and occasionally during emergency (trauma) surgery. Given the spleens role in the immunological response, overwhelming infection remains an area of concern for post-splenectomy patients with low prevalence (0.1-0.5%) but high mortality (∼50%). Strep pneumoniae, H influenzae Type B and Neisseria meningitidis are common bacterial perpetrators. At present, Public Health England and the British Society for Haematology recommends regular influenza, pneumococcal and meningococcal vaccinations for patients with asplenia or hyposplenism. They should also be on lifelong prophylactic antibiotics. This audit aimed to assess the adherence to vaccination and antibiotic prophylaxis guidelines for post-splenectomy patients. Methods 19 patients post splenectomy patients were identified at a primary care practice in North West England. Data was retrospectively collected from the last 10 years with two researchers manually searching through their GP records. Parameters collected included both compliance and timeliness with vaccinations and antibiotic prophylaxis as per national guidance. Where there was a deviation from the guidance, the records were again searched to identify any cause. Results 109/162 (67.3%) ‘vaccination opportunities’ for the recommended yearly influenza vaccination were successfully delivered. 17/19 (89.4%) patients had no documented initial pneumococcal vaccination at time of splenectomy. 23/162 (14.2%) influenza and 22/37 (56.4%) pneumococcal vaccination opportunities were missed with no documentation explaining why. Only 1 patient received the recommended MenACWY/MenB vaccination at diagnosis, 5 received delayed vaccinations and 13/19 (68.4%) patients received no meningitis vaccination. 7/19 (37%) patients were not on prophylactic antibiotics as recommended with no documented explanation. 2 of those patients were issued one-off antibiotic prescriptions and 1 had their prescription stopped due to non-compliance. Conclusions Significant improvement is needed with regards to antibiotic prophylaxis, pneumococcal and meningococcal vaccinations with over 50% of vaccinations being missed. Poor documentation and a shift to electronic health records are possible factors that have affected data collection, particularly as a significant number of the splenectomies performed were prior to 2000. Recommendations include routine reviews for asplenic patients, encouragement for opportunistic vaccination and increasing awareness of the risks of post-splenectomy sepsis and the benefits of vaccinations. Future scope could include looking at the same guidelines for other hyposplenic/asplenic conditions.

Angioembolization for Isolated Severe Blunt Splenic Injuries with Hemodynamic Instability: A Propensity Score Matched Analysis.

This study aimed to compare patient outcomes after splenic angioembolization (SAE) or splenectomy for isolated severe blunt splenic injury (BSI) with hemodynamic instability, and to identify potential candidates for SAE. Adult patients with isolated severe BSI (Abbreviated Injury Scale [AIS] 3-5) and hemodynamic instability between 2013 and 2019 were identified from the American College of Surgeons Trauma Quality Improvement (ACS TQIP) database. Hemodynamic instability was defined as an initial systolic blood pressure (SBP) <90mmHg, heart rate (HR) >120bpm, or lowest SBP <90mmHg within 1h after admission, with ≥1 unit of blood transfused within 4h after admission. In-hospital mortality was compared between splenectomy and SAE groups using 2:1 propensity-score matching. The characteristics of unmatched and matched splenectomy patients were also compared. A total of 478 patients met our inclusion criteria (332 splenectomy, 146 SAE). After propensity-score matching, 166 splenectomy and 83 SAE patients were compared. Approximately 85% of propensity-score matched patients sustained AIS 3/4 injuries, and 50% presented with normal SBP and HR before becoming hemodynamically unstable. The median time to intervention (splenectomy or SAE) was 137min (interquartile range 94-183). In-hospital mortality between splenectomy and SAE groups was not significantly different (5.4% vs. 4.8%, p = 1.000). More than half of unmatched patients in the splenectomy group sustained AIS 5 injuries and presented with initially unstable hemodynamics. The median time to splenectomy in such patients was significantly shorter than in matched splenectomy patients (67 vs. 132min, p < 0.001). Splenectomy remains the mainstay of treatment for patients with AIS 5 BSI who present to hospital with hemodynamic instability. However, SAE might be a feasible alternative for patients with AIS 3/4 injuries.

Impact of splenectomy on circulating microparticles in patients with sickle cell anemia.

Circulating microparticles (MP) are being described as potential biomarkers for disease activity in a variety of conditions including sickle cell anemia (SCA). However, relatively little is known about the influence of spleen status on MP levels in patients with SCA. Using a prospective study design we characterize circulating MP in 144 patients with SCA in steady state by assessing their cellular origin and their relationships to spleen status defined by clinical and imaging findings. In addition, MP levels were studied according to demographic characteristics, clinical status, treatment modalities, and other hematological and biochemical parameters. Absolute plasma concentrations of MP were determined by flow cytometry. Patients with SCA displayed a 10-fold increase in levels of MP derived from red blood cell (RBC) and platelets (PLT) when compared to their healthy counterparts (p < 0.0001). Splenectomized patients with SCA have more pronounced levels of MPRBC and MPPLT, and remained elevated after several weeks of follow-up. Levels of MP were not significantly associated with spleen removal procedures, age, gender, clinical severity score, hydroxyurea therapy, hemoglobin F, and co-existence of glucose-6-phosphate dehydrogenase deficiency. Collectively, these results suggest that splenectomy affects circulating levels of MP regardless of the known SCA modifiers and correlates.

Assessment of the potential role of Trefoil Factor-3 marker as a predictive marker of complication in splenectomized and non splenectomized patients with beta thalassemia major

Abstract The study’s goal is to appraise the immunological inflammatory marker Trefoil Factor 3, which interacts with thalassemia pathogenesis particularly following splenectomy, and may offer new therapy options for the illness and its repercussions. This is a case-control study design that included 60 patients identified as β-thalassemia major as participators in this study, in addition to 30 seemingly healthy subjects with age and sex close to the patients group who served as a control group. The participants were distributed into four groups: control group, splenectomized patients, non-splenectomized patients, and total patients. Suitable statistical techniques were employed to investigate the results. The study’s findings demonstrated that there was a significance increase in the serum levels of TFF3 when comparing between (splenectomized, non-splenectomized and total patients) with healthy group (322.16±51.241, p-value=0.01, 317.20±42.449, p-value=0.01, 320±46.6, p-value=0.01), vs (309.38±21.94), respectively. Moreover, a comparison between splenectomized and non-splenectomized showed a significantly decrease in TFF3 (322.16±51.241) vs (317.20±42.449), (p-value=0.043).The presented study also revealed significant positive correlation between TFF3 level with ferritin, iron, total iron binding capacity, transferrin saturation, transferrin, fasting serum glucose, insulin and homeostasis model assessment-insulin resistance. Furthermore, unsaturated iron binding capacity and homeostasis model assessment-beta found a significant negative correlation with TFF3 level. High serum levels of TFF3 in beta thalassemia patients, especially in splenectomies patients, are downregulated by inflammatory cytokines, which are primarily regarded as traditional inflammatory cytokines and are related to insulin resistance. Hence, TFF3 level can serve as a potential predictive for the early detection of beta thalassemia in the development and progression of complications.

Splenectomy improves erythrocyte functionality in spherocytosis based on septin abundance, but not maturation defects.

Splenectomy improves the clinical parameters of patients with hereditary spherocytosis, but its potential benefit to red blood cell (RBC) functionality and the mechanism behind this benefit remain largely overlooked. Here, we compared 7 nonsplenectomized and 13 splenectomized patients with mutations in the β-spectrin or the ankyrin gene. We showed that hematological parameters, spherocyte abundance, osmotic fragility, intracellular calcium, and extracellular vesicle release were largely but not completely restored by splenectomy, whereas cryohemolysis was not. Affected RBCs exhibited decreases in β-spectrin and/or ankyrin contents and slight alterations in spectrin membrane distribution, depending on the mutation. These modifications were found in both splenectomized and nonsplenectomized patients and poorly correlated with RBC functionality alteration, suggesting additional impairments. Accordingly, we found an increased abundance of septins, small guanosine triphosphate-binding cytoskeletal proteins. Septins-2, -7, and -8 but not -11 were less abundant upon splenectomy and correlated with the disease severity. Septin-2 membrane association was confirmed by immunolabeling. Except for cryohemolysis, all parameters of RBC morphology and functionality correlated with septin abundance. The increased septin content might result from RBC maturation defects, as evidenced by (1) the decreased protein 4.2 and Rh-associated glycoprotein content in all patient RBCs, (2) increased endoplasmic reticulum remnants and endocytosis proteins in nonsplenectomized patients, and (3) increased lysosomal and mitochondrial remnants in splenectomized patients. Our study paves the way for a better understanding of the involvement of septins in RBC membrane biophysical properties. In addition, the lack of restoration of septin-independent cryohemolysis by splenectomy may call into question its recommendation in specific cases.

Correlates of splenic T2* measurements in transfusion-dependent thalassaemia and comparison between non-splenectomised and splenectomised patients.

Correlates of splenic T2* measurements in transfusion-dependent thalassaemia and comparison between non-splenectomised and splenectomised patients.

Time Trend Analysis of Splenectomy for Splenic Marginal Zone Lymphoma: Declining Surgery, Promising Survival.

The purpose of current study was to examine the incidence, characteristics, treatment, and survival of splenic marginal zone lymphoma (SMZL). Using SEER-18 database, patients diagnosed with SMZL between 2000 and 2018 were included. Effect of splenectomy on survival was evaluated after balancing the confounding factors by propensity score matching. Rates of splenectomy and 1-year relative survival were calculated for each year. A logistic regression model identified factors related to splenectomy, and a Cox regression model assessed factors linked to overall survival (OS). A total of 2790 patients with SMZL were analyzed. The majority were older than 60 years, female, and white. The age-adjusted incidence of SMZL was 0.17/100,000 person-years, with higher incidence in males. Incidence increased by 0.68%/year and peaked at 80-84 years for both genders. The SMZL-specific survival rates at 3 and 5 years were 89.6% and 85.3%, respectively. Meanwhile, the relative survival rates for the same periods were 88.6% and 85.9%, respectively. Splenectomy patients were more likely to be younger, male, and diagnosed with early-stage disease. Despite the decreasing utilization rate of splenectomy from 59.4% in 2000 to 16.2% in 2018, the 1-year relative survival rate remained relatively stable with minor fluctuations over time. Whether or not the patient underwent splenectomy was not found to be a significant prognostic indicator for OS. Our study demonstrated a decreasing use of splenectomy but a relatively stable survival in patients with SMZL, highlighting the urgency to better understand the role of splenectomy and its associated outcomes.

Different Surgeon, Different Closure: Lack of Consensus on Appropriate Closure Technique for Various Case Scenarios.

Background: Many techniques for closure of surgical incisions are available to the surgeon, but there is minimal guidance regarding which technique(s) should be utilized at the conclusion of surgery and under what circumstances. Hypothesis: Management of incisions at the conclusion of surgery lacks consensus and varies among individual surgeons. Methods: The Surgical Infection Society membership was surveyed on the management of incisions at the conclusion of surgery. Several case scenarios were provided to test the influences of operation type, intra-operative contamination, and hemodynamic stability on incision management (e.g., close fascia or skin, use of incision/wound vacuum-assisted closure [VAC] device). Responses by two-thirds of participants were required to achieve consensus. Data analysis by χ2 test and logistic regression, a = 0.05. Response heterogeneity was quantified by the Shannon index (SI). Results: Among 78 respondents, consensus was achieved for elective splenectomy (91% close skin/dry dressing). Open appendectomy and left colectomy/end-colostomy had the greatest heterogeneity (SI, 1.68 and 1.63, respectively). During trauma laparotomy, the majority used damage control for hemodynamic instability (53%-67%) but not for hemodynamically stable patients (0%-1.3%; p < 0.001). Additional consensus was achieved for close skin/dry dressing for hemodynamically stable trauma splenectomy patients (87%) and fascia open/wound VAC for hemodynamically unstable colon resection/anastomosis (67%). Fecal diversion for rectal injury and colon resection/anastomosis (both when hemodynamically stable) had high heterogeneity (SI, 1.56 and 1.48, respectively). In penetrating trauma, sentiment was for more use of wet-to-dry dressings and incision/wound VAC with increased contamination in hemodynamically stable patients. Conclusions: Damage control was favored in hemodynamically unstable trauma patients, with use of wet-to-dry dressings and incision/wound VAC with spillage after penetrating trauma. However, most scenarios did not achieve consensus. High variability of practices regarding incision management at the conclusion of surgery was confirmed. Prospective studies and evidence-based guidance are needed to guide decision making at end-operation.

Assessment of serum fractalkine level as potential role for complications in splenectomized and non-splenectomized patients with β-thalassemia major

Introduction: Thalassemia is a serious genetic blood related condition characterized by a lack of or malfunctioning hemoglobin in the RBCs. One of the most widespread inherited disorders is beta thalassemia (β-TM). The high susceptibility to infection in these individuals' is related to high mortality and morbidity rates. Fractalkine is known as CX3C chemokine ligand 1 (CX3CL1), which contains a total of 371 amino acid, and participates in a number of functions, including repair of tissues after injury, immunological response during inflammation, and chemotaxis effet.&#x0D; Methodology: A case control study, containing sixty patients recognized as suffering from β-TM, was conducted at Al Zahra Teaching Hospital, Najaf (Iraq). The patients were divided into two groups; splenectomized and non-splenectomized patients. The thalassemia disease was enrolled in the “Thalassemia Unit”. Suitable statistical techniques were used to analyze the outcomes.&#x0D; Results: In the current study, a significant increase in serum level of CX3CL1 was found in patients with β-TM, especially in splenectomized group (14.914 ± 2.636), (P = 0.01) and non-splenectomy (13.816 ± 2.686), (P = 0.05) group as compared with control group (12.26 ± 1.797).The linear regression analysis showed that a significant positive correlation in ferritin with serum CX3CL1 level in splenectomy patients with β-TM group. ROC curve for CX3CL1 that might be diagnosis of patients with β-TM with an AUC of 0.757 (95% CI: 0.623-0.890), sensitivity and specificity equally 75% and 83.3%.&#x0D; Conclusion: Increase level of CX3CL1 due to its interaction with CX3CR1 receptor may lead to buildup of inflammatory cells in the β-TM patients, blocking CX3CL1 signaling may be helpful for patients with β-TM.&#x0D; Abbreviations: AUC – Area Under Curve; β-TM - Beta Thalassemia; ROC - Receiver Operating Characteristic UIBC - Unsaturated Iron-Binding Capacity test; TIBC - Total Iron-Binding Capacity&#x0D; Key words: Thalassemia, β thalassemia major, Fractalkine&#x0D; Citation: Awad AS, Ali HA, Althalmi SAA. Assessment of serum fractalkine level as potential role for complications in splenectomized and non-splenectomized patients with β-thalassemia. Anaesth. pain intensive care 2023;27(5):495−500; DOI: 10.35975/apic.v27i5.2302&#x0D; Received: August 03, 2023; Reviewed: August 12, 2023; Accepted: August 14, 2023

The potential benefits and risks of sex-steroids therapy in thalassemic patients with hypogonadism

Introduction: A recent review from 14 Mediterranean and Middle East countries (n =4477, mean age = 16.5 years) showed that the pooled prevalence of delayed puberty / hypogonadism in patients with BTM was 45.6%. Studying the consequences of hypogonadism and the potential benefits versus unwanted side effects of sex-steroid hormone replacement therapy (HRT) in children and adolescents with Beta Thalassemia Major (BTM) represent an important issue for these patients. Objectives: We reviewed the literature (Pubmed, Google scholar, Scopus, Research gate) (1995: 2022) on the consequences of hypogonadism and benefits versus side effects of using sex Steroid therapy (HRT) in hypogonadal adolescents with BTM. Thirty-five papers were included and analyzed. Results: HRT had a variable but significant beneficial effect on improving bone mineral density (BMD), pubertal growth spurt, development of secondary sexual characteristics and subsequently improved quality of life (QOL) in children and adolescents with BTM. Increased side effects of HRT in these patients may occur due to their state of hypercoagulability especially in splenectomized patients and those with cardiomyopathy, chronic hepatitis, and dysglycemia. Gynecomastia and local pain in the site of injection confirmed in a considerable number of males on testosterone therapy. Local reaction to sex steroid patches appeared in around 30% of patients. Based on this review recommendations for using HRT in these patients were updated. Conclusion: Proper and wise use of sex steroid therapy in patients with BTM can significantly improve many consequences of hypogonadism.

Relation of Immunoglobulin Level and White Blood Cell Count with Frequency of Infection in Splenectomized and Non-Splenectomized B Thalassemia Major Patients

Background: Beta-thalassemia major is an autosomal recessive condition caused by absent (β0) synthesis of the β globin chains of the hemoglobin tetramer. Objectives: Evaluation of immunoglobulin level and white blood cell count in splenectomized and non non splenectomized patient as well as comparison of the levels of Ig and WBC with frequency of infection. Materials and Methods: This cross sectional study included a total of 60 patients with beta-thalassemia major and 20 age and sex matched apparently healthy individual as a control, blood taken from them for an evaluation of immunoglobulin level and white blood cell count. Results: There is no significant difference in immunoglobulin level between splenectomized and non splenectomized B-thalassemia major patients and control groups. Also, there is significant increase in mean Frequency of infection / year for patients with thalassemia major in comparison to control, Specifically, splenectomized patients are having significant increase in frequency of infection in comparison to non splenectomizd. There is a significant difference in mean of White blood cell count, Neutrophil, Lymphocytes in patients with splenectomy, non-splenectomy and control group. Conclusion: Immunoglobulin levels are within normal range in thalassemia major patients whether splenectomized or non splenectomized. Leukocytes count mainly neutrophils and lymphocytes are higher in splenectomised patients. Frequency of infection is higher in splenectomed patients and there is a positive correlation with IgA and IgG.

Liver manifestations of tick-borne diseases.

Liver manifestations of tick-borne diseases.

Geographic disparities in the care and outcomes in adult chronic immune thrombocytopenia

IntroductionDespite expert-based recommendations, real-world adherence to immune thrombocytopenia (ITP) guidelines is unclear. The impact of geographic and socioeconomic disparities on the quality of care and outcomes is unknown. We sought to determine the association between geographic remoteness and material deprivation on ITP care and outcomes. MethodsWe conducted a multi-centre retrospective cohort study of adults with chronic ITP requiring a second-line therapy between 2012 and 2019 in the province of Alberta, Canada. Socioeconomic status was measured using the Pampalon material deprivation index quintiles. Geographic disparities were assessed by the driving distance to a major centre, with geographic remoteness defined as >200 km from major centre. We examined the impact of geographic and material deprivation on quality of care, resource utilization (hospitalizations, transfusions), and outcomes (major bleeding, all-cause mortality and ITP-related mortality). Cox proportional hazards models were used to examine the impact of geographic remoteness, rural residence and material deprivation on overall survival and ITP-related survival. ResultsWe included 326 ITP patients, median age of ITP diagnosis was 57 years, 182 (56 %) were female. Most patients (58 %) lived within 20 km of a major centre, whereas 49 (15 %) lived in a geographically remote area (>200 km). Geographic remoteness was significantly associated with material deprivation and lower likelihood of management by hematologists (84 % vs 99 %, P = 0.0001). It was also associated with lower rates of hepatitis C (71 % vs 89 %, P = 0.005) and hepatitis B testing (69 % vs 86 %, P = 0.03), and a non-significant trend towards lower rates of HIV testing (73 % vs 83 %, P = 0.051) compared with those <20 km from a major centre. Incomplete hyposplenic vaccinations among splenectomized patients (52 %), early splenectomy within 12 months of ITP diagnosis (35 %), inappropriate platelet transfusions (41 %), and inappropriate hospitalizations for asymptomatic thrombocytopenia (16 %) were common regardless of geographic distribution. There were 28 (9 %) ITP-related deaths (major bleeding or infections), most occurred within the first year of ITP diagnosis. Material deprivation, but not geographic remoteness, was an independent predictor of all-cause mortality (aHR 1.9, 95 % CI 1.1–3.3 in the most deprived quintile vs least deprived quintile). Rural residence trended towards increased hazard of ITP-related deaths (aHR 1.7, 95 % CI 0.9–3.2). ConclusionWe demonstrated substantial deviations of ITP care from consensus guidelines, and geographic disparities in access to care and diagnostic workup. Future quality improvement initiatives are critical to improve the quality of care and reduce inequities.