Cases Of Immune Thrombocytopenia Research Articles

Romiplostim use in immune thrombocytopenia: Experience in Cuenca, Ecuador

Introduction. The international consensus and the American Society of Hematology guidelines from 2019 established thrombopoietin analogues as the second-line therapy for primary immune thrombocytopenia cases. Objectives. To describe romiplostim usefulness in patients with immune thrombocytopenia in a third-level hospital in Cuenca, Ecuador. Materials and methods. We conducted a descriptive and retrospective study in patients with immune thrombocytopenia treated with romiplostim. We evaluated the following variables: age, gender, previous therapies to romiplostim, dose, frequency, complications, change of thrombopoietin analogue, and treatment discontinuation. Results. We included 21 patients with immune thrombocytopenia treated with romiplostim, with a median age of 49 years. All patients received corticosteroids as first-line treatment. Three patients required longer administration intervals (over a week), with weekly doses lower than those recommended (< 1 μg/kg). Due to lack of efficacy, six patients replaced elthrombopag with romiplostim. Of the total, three suffered thrombotic complications: two had portal venous thrombosis, and one had pulmonary thromboembolism; five of the patients discontinued romiplostim scheme without resuming it. Conclusions. Romiplostim constitutes a convenient second-line therapy in immune thrombocytopenia. Despite the small sample size, romiplostim early use can minimize toxicities and infectious risks.

PD-1/PD-L1 inhibitor-induced immune thrombocytopenia: A pharmacovigilance study and systematic review

IntroductionProgrammed cell death-1 (PD-1) and programmed cell death ligand-1 (PD-L1) immune checkpoint inhibitors (ICIs) are used for a variety of cancers and are associated with a risk of developing immune-related adverse events, most commonly dermatitis, colitis, hepatitis, and pneumonitis. Immune-mediated hematologic toxicities have been reported, but are less well-described in the literature. Immune thrombocytopenia (ITP) is a rare autoimmune, hematologic adverse event that has been reported with PD-1/PD-L1 inhibitors. MethodsWe performed a retrospective observational analysis of the United States Food and Drug Administration Adverse Event Reporting System (FAERS) data. We searched for cases of ITP reported with exposure to PD-1/PD-L1 inhibitors from initial FDA approval for each agent to September 30, 2022. Disproportionality signal analysis was done by calculating the reporting odds ratio (ROR). Oxaliplatin was used as a positive control for sensitivity analysis as it is an anticancer therapy that has been associated with drug-induced ITP. A systematic review of the PubMed database was also conducted to identify published cases of PD-1/PD-L1 inhibitor-induced ITP. ResultsThere were 329 reports of ITP with ICIs in the FAERS database that were reviewed for a disproportionality signal, including atezolizumab (n = 27), durvalumab (n = 17), nivolumab (n = 160), and pembrolizumab (n = 125). The ROR was significant for atezolizumab (ROR 5.39, 95 % CI 3.69–7.87), avelumab (ROR 10.32, 95 % CI 4.91–21.69), durvalumab (ROR 7.91, 95 % CI 4.91–12.75), nivolumab (ROR 9.76, 95 % CI 8.34–11.43), and pembrolizumab (ROR 12.6, 95 % CI 10.55–15.06). In our systematic review, we summated 57 cases of ICI-induced ITP. Nivolumab and pembrolizumab had the most reported cases of ITP in the literature. Most cases reported (53 %) included ITP-directed therapies beyond corticosteroids for the management of ICI-induced ITP. ConclusionThere is a significant reporting signal of ITP with several ICI agents. Clinicians should be aware of and monitor for signs of this potentially serious adverse event.

Clinical significance of platelet membrane glycoproteins in children with primary immune thrombocytopenia

Abstract Objectives To measure CD62p and CD42b levels and to study its correlations with bleeding score, and different prognostic factors in children with primary immune thrombocytopenia (ITP) patients. Methods In all, 60 children with ITP and 30 age- and sex-matched healthy participants were recruited and underwent thorough clinical assessments including bleeding score using the modified Buchanan and Adix bleeding score as well as complete blood count with measurement of mean platelet volume (MPV), platelet distribution width (PDW) and plateletcrit (PCT), coagulation profiles and measurement of platelet glycoproteins (CD62p and CD42b) using a flow cytometer. Results Children with ITP had lower PCT (P&lt;0.001), CD 62 P, and co-expression of CD 42b/CD 62 P and higher MPV (P=0.009), PDW (P&lt;0.001), and CD 42b level. MPV correlates negatively with lymphocyte count and platelet count, while PDW correlates negatively with neutrophil count and positively with hemoglobin. PCT correlates negatively with total leucocyte count, neutrophil count, and positively with platelet count (P=&lt;0.001). Children with chronic ITP usually presented with mild cutaneous bleeding, while those with acute ITP usually suffered from severe mucosal and life-threatening bleeding (P=0.001). Acute ITP cases had the highest total leucocyte count (P&lt;0.001) and the lowest count of platelet (P&lt;0.001); persistent cases had the lowest lymphocyte count (P=0.023) and co-expression of CD42b/CD62p (P=0.039), while chronic cases had the lowest neutrophil count P&lt;0.001) and highest percentage of PCT (P&lt;0.001) and co-expression of CD42b/CD62p (P=0.039). Conclusion Platelet parameters and platelet glycoprotein CD62P and CD42b expression is a simple noninvasive diagnostic tool in ITP children.

Good Prognosis of Adult Immune Thrombocytopenia in a Provincial Hospital

Background: Immune thrombocytopenia (ITP) is an autoimmune disease resulting from platelet destruction and impaired platelet production. Some patients experience relapses or refractoriness after corticosteroid treatment, necessitating second-line treatments. The majority of adult ITP cases become chronic or relapsing. The aim of this study was to assess the clinical course and identify factors related to sustained responses to treatment in patients with ITP. Methods: This observational retrospective cohort study was conducted from January 2013 to December 2022 at Trang Hospital, Trang Province, Thailand. Results: A total of 125 newly diagnosed ITP patients were enrolled, with an average follow-up duration of 48.49 ± 41.49 (mean ± SD) months. The average age was 42.34 ± 18.44 years, the average platelet count was 11.66 ± 14.39 x 10 9 /L, and the average platelet recovery time after treatment was 43.14 ± 101.32 days. The mean response duration after the first-line treatment was 25.99 ± 29.39 months. The characteristics of the patients were as follows: age below 60 years (78.4%), female sex (75.2%), pregnancy (1.6%), primary ITP (67.2%), secondary ITP (32.8%), bleeding symptoms (54.4%), platelet count &amp;lt;10 x 10 9 /L (64%), platelet count 10-30 x 10 9 /L (28.8%), platelet count 30-50 x 10 9 /L (5.6%), platelet count &amp;gt;50 x 10 9 /L (1.6%), splenectomy (4%), observation only (2.4%), loss to follow-up (10.4%), and death (4%). The most common cause of secondary ITP was systemic lupus erythematosus (63%). The first-line treatment consisted of dexamethasone (70.4%), prednisolone (23.2%), and/or intravenous immunoglobulin (4%). The clinical courses were as follows: recovery within 3 months in 68.8%, persistence in 13.6%, and chronic ITP in 17.6%. The sustained responses to corticosteroid treatment at 6 months included complete response (CR) (58.4%) and response (R) (9.6%), as shown in Table 1. Second-line treatments were required in 22.4% of cases, including colchicine (60.7%), azathioprine (35.7%), cyclophosphamide (25%), dapsone (17.8%), vincristine (10.7%), mycophenolate mofetil (7.1%), cyclosporine (3.5%), and/or eltrombopag (3.5%). The sustained responses to second-line treatments at 6 months were CR in 12% and R in 8.8%. Female sex ( P-value 0.007) and primary ITP ( P-value 0.046) were significantly associated with the sustained response (CR+R) to corticosteroids at 6 months. Secondary ITP ( P-value 0.036) was also associated with the sustained response (CR+R) to second-line treatments at 6 months (Table 2). Conclusions: This study reveals a favorable prognosis for ITP, with a majority of patients showing sustained response (CR+R), which differs from findings in studies conducted at referral centers. Female patients and those with primary ITP are more likely to respond. Further confirmation is needed through a large prospective cohort study in community hospitals.

Atrial Fibrillation Patients with Immune Thrombocytopenia: A Review of Management Options and Bleeding Risks

Aim : The objective of this research is to review the bleeding risks in patients with atrial fibrillation (Afib) and immune thrombocytopenia (ITP) and explore various management strategies to formulate an optimal therapeutic approach that improves the prognosis of these patients. Methods: In this study, we devised our search approach by utilizing PubMed's Medical Subject Headings (MeSH) terms and incorporating pertinent keywords extracted from article titles and abstracts. To ensure a comprehensive scope, we integrated terms associated with immune thrombocytopenia purpura, such as “Immune Thrombocytopenia,” and similar expressions. Additionally, terms related to atrial fibrillation, including “atrial fibrillation,” were included to identify articles discussing atrial fibrillation in the context of immune thrombocytopenia. To adapt the initial search strategy for Embase, Web of Science, and Scopus databases, we employed a polyglot translator. All identified studies resulting from the search strategy were imported into EndNote, where duplicate articles were meticulously removed. The remaining studies were then transferred to Rayyan for further duplicate elimination and to initiate the screening process. The study incorporates full-text articles, submitted abstracts, and conference abstracts. Excluded from this analysis were studies falling into the following categories: (1) animal studies, (2) reviews or non-original articles, and (3) non-English articles. Results: A total of 1891 articles were retrieved from four databases, and after careful selection, 14 relevant full-text articles were analyzed. Among these, 12 studies investigated the efficacy of anticoagulants in managing cases of concurrent Afib and ITP as well as their bleeding risks. Comparing bleeding risk between groups was usually done through measuring by CHA 2DS 2 VASc or HAS BLED scoring systems and survival was also compared by using Kaplan-Meier curves A few of them also compared the efficacy and safety of warfarin against Non-Vitamin K Antagonists which has shown that Non-Vitamin K Antagonists have a lower event rate of major bleeding, lower hazard ration of systemic embolism and higher survival rate compared to warfarin The remaining two papers explored the efficacy and safety of surgical interventions, specifically left atrial appendage closure. Many studies reported that anticoagulant therapies were associated with reduced bleeding risk and have shown promising outcomes in managing patients with Afib and ITP. Additionally, percutaneous left atrial appendage closure has been reported to be a potentially safe management option for atrial fibrillation in patients with primary hemostatic disorders, including thrombocytopenia. Conclusion: In essence, this review focused on investigating the bleeding risk associated with atrial fibrillation (Afib) in patients with immune thrombocytopenia (ITP) and examining available management strategies. While the precise pathophysiological mechanisms linking atrial fibrillation and ITP remain incompletely understood, the review emphasized the intricate interplay between thromboembolic risk and bleeding complications in this specific patient group. The findings suggest that certain anticoagulation regimens and also left atrial appendage closure have shown promising safety and effectiveness in Afib patients with thrombocytopenia. Nevertheless, further research with larger sample sizes and diverse populations is needed to enhance our understanding of the Afib-ITP relationship. Such studies will yield more definitive conclusions and valuable insights into optimal anticoagulation approaches and alternative therapies for patients with both Afib and ITP.

A Novel Online Decision Support Tool to Determine Practice Patterns and Concordance with Experts in the Treatment of Patients with Immune Thrombocytopenia

Background: Immune thrombocytopenia (ITP) is a complex disease involving dysregulation of the immune system with inadequate platelet production and increased platelet destruction. When treatment is warranted, corticosteroids are frequently used as first-line therapy. Steroid-refractory patients eventually require alternative treatments, of which there are several approved and well-studied options. However, these second-line treatments have not been rigorously compared in clinical trials. To assist healthcare professionals (HCPs) in managing patients with ITP, we developed an online interactive decision support tool to provide expert recommendations for treatment. Here we report an analysis of practice patterns among HCPs and experts for ITP cases after initial therapy failure and the impact of expert recommendations on HCP decisions. Methods: An online tool was developed with 4 experts providing recommendations on management of 332 unique patient cases based on patient- and disease-specific factors that the experts considered relevant to making treatment decisions. HCPs entered patient case details including age, treatment setting, platelet count, current presentation, and presence of risk factors for bleeding, along with their intended management approach, before being shown the expert recommendations for their specific scenario. Results: Between October 2022 and July 2023, 311 patient cases were entered by 256 HCPs, including 194 (62.4%) cases of treatment-exposed patients (Table). Of the treatment-exposed cases, 72.1% were submitted by physicians, and 23.2% were submitted by HCPs in North America. There was strong alignment between HCP treatment selection and at least 1 expert recommendation (69%; P = .0001). There was consensus among experts for 94.4% of cases with patients ≥21 years of age who were treatment exposed. In these cases, experts recommended thrombopoietin receptor agonists (TPO-RA) for 83.5% of cases in contrast to HCPs recommending them in 38.2% of cases. Alternatives for TPO-RA chosen by HCPs in cases with patients ≥21 years of age who were treatment exposed included rituximab (29.6%), immunoglobulin (23.5%), fostamatinib (12.5%), splenectomy (9.8%), observation (8.6%), or unsure (16%). There was consensus among experts for 72% of cases with patients &amp;lt;21 years of age who were treatment exposed. For cases with patients &amp;lt;21 of age who were treatment exposed, HCPs chose immunoglobulin (28.6%), observation (28.6%), rituximab (14.3%), splenectomy (4.7%), or unsure (23.8%). In 47.6% of cases where HCPs indicated an alternative selection, they indicated that expert recommendations changed their treatment choice. HCPs indicated there were barriers to implementation in 7% of indicated cases. Conclusion: Based on our analysis of an online treatment decision tool, practice patterns for the management of patients with ITP aligned between HCPs and at least 1 tool expert in 69% of treatment-experienced cases. For previously treated patients, HCPs were less likely to choose a TPO-RA compared with tool experts, although very few identified barriers to implementation. Although specific treatment utilization patterns differed between HCPs and experts based on data from this tool, HCPs appeared amenable to practice changes based on expert recommendations. Online decision support tools can offer opportunities for HCPs to receive education and expert guidance in choosing optimal treatment regimens for ITP, a condition where treatment guidelines may not address every unique patient scenario.

PB1351 High-Doses of Dexamethasone as an Alternative to Other Steroidal Use in Primary Immune Thrombocytopenia Cases: A Research Study in a Salvadoran Population

PB1351 High-Doses of Dexamethasone as an Alternative to Other Steroidal Use in Primary Immune Thrombocytopenia Cases: A Research Study in a Salvadoran Population

Cytomegalovirus Infection-related Immune Thrombocytopenia: A Case Report

Immune thrombocytopenia (ITP) is an acquired cause of thrombocytopenia characterized by the presence of autoantibodies against platelets. It may be primary or secondary to several conditions. We present the case of a 63-year-old woman with a diagnosis of immune thrombocytopenia refractory to conventional therapy. After she was tested for secondary causes of ITP, a diagnosis of acute cytomegalovirus (CMV) infection was made. She was treated with ganciclovir and presented normalization of platelet count. CMV-related Immune Thrombocytopenia should always be considered in certain cases of refractory ITP. If the diagnosis of ITP secondary to acute CMV infection is made, specific antiviral therapy with ganciclovir should be considered. In these cases, immunosuppressive agents, such as steroids, may worsen the ITP and should be tapered or withdrawn as rapidly as feasible.

Pediatrik İmmün Trombositopeni (İTP) Vakalarının ve Kronik İTP için Risk Faktörlerinin Değerlendirilmesi - Tek Merkez Deneyimi

Aim: Immune thrombocytopenia (ITP) is the most common acquired bleeding disorder in childhood. The study aimed to assess the demographic and clinical characteristics, and treatment responses and to evaluate their effects on chronicity in pediatric ITP cases.&#x0D; Material and Methods: Primary ITP patients aged 1 month to 18 years, who were diagnosed and followed up in the Pediatrics Clinic of Tokat Gaziosmanpaşa University Hospital between January 2010 and December 2018, were retrospectively analyzed.&#x0D; Results: Thirty-eight patients with a diagnosis of primary ITP were included in the study. The mean age of the patients was 94.3±53.4 (14-199) months. The female/male ratio was 1. Twenty (57.1%) patients had acute ITP, and 15 (42.9%) patients had chronic ITP. There was no significant difference between the acute ITP group and the chronic ITP group in demographic, clinical features, laboratory findings, and treatment responses. In the first 12 months, the number of admissions with a platelet count of

Early initiation of second-line therapy in primary immune thrombocytopenia: insights from real-world evidence

To compare patients with primary immune thrombocytopenia (ITP) prescribed early (within 3 months of initial ITP treatment) second-line treatment (eltrombopag, romiplostim, rituximab, immunosuppressive agents, splenectomy) with or without concomitant first-line therapy to those who received only first-line therapy. This real-world retrospective cohort study of 8268 patients with primary ITP from a large US-based database (Optum® de-identified Electronic Health Record [EHR] dataset) combined electronic claims and EHR data. Outcomes included platelet count, bleeding events, and corticosteroid exposure 3 to 6 months after initial treatment. Baseline platelet counts were lower in patients receiving early second-line therapy (10‒28 × 109/L) versus those who did not (67 × 109/L). Counts improved and bleeding events decreased from baseline in all treatment groups 3 to 6 months after the start of therapy. Among the very few patients for whom follow-up treatment data were available (n = 94), corticosteroid use was reduced during the 3- to 6-month follow-up period in patients who received early second-line therapy versus those who did not (39% vs 87%, p < 0.001). Early second-line treatment was prescribed for more severe cases of ITP and appeared to be associated with improved platelet counts and bleeding outcomes 3 to 6 months after initial therapy. Early second-line therapy also appeared to reduce corticosteroid use after 3 months, although the small number of patients with follow-up data on treatment precludes any substantive conclusions. Further research is needed to determine whether early second-line therapy has an effect on the long-term course of ITP.

Genome-wide DNA methylation profiling of CD4+ T lymphocytes identifies differentially methylated loci associated with adult primary refractory immune thrombocytopenia

BackgroundDNA methylation played a crucial role in the pathogenesis of immune thrombocytopenia (ITP). However, genome-wide DNA methylation analysis has not been applied thus far. The present study aimed to provide the first DNA methylation profiling for ITP.MethodsPeripheral blood CD4+ T lymphocytes samples were collected from 4 primary refractory ITP cases and 4 age-matched healthy controls, and DNA methylome profiling was performed using Infinium MethylationEPIC BeadChip. Differentially methylated CpG sites were further validated in another independent cohort of 10 ITP patients and 10 healthy controls using qRT-PCR.ResultsThe DNA methylome profiling identified a total of 260 differentially methylated CpG sites mapping to 72 hypermethylated and 64 hypomethylated genes. These genes were mainly enriched in the actin nucleation of the Arp2/3 complex, vesicle transport, histone H3-K36 demethylation, Th1 and Th2 cell differentiation, and Notch signaling pathway according to the GO and KEGG databases. The mRNA expression of CASP9, C1orf109, and AMD1 were significantly different.ConclusionsGiven the altered DNA methylation profiling of ITP, our study provides new insights into its genetic mechanism and suggests candidate biomarkers for the diagnosis and treatment of ITP.

Analysis of hematologic adverse events reported to a national surveillance system following COVID-19 bivalent booster vaccination.

Hematologic complications, including vaccine-induced immune thrombotic thrombocytopenia (VITT), immune thrombocytopenia (ITP), and autoimmune hemolytic anemia (AIHA), have been associated with the original severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines. However, on August 31, 2022, new formulations of the Pfizer-BioNTech and Moderna vaccines were approved for use without clinical trial testing. Thus, any potential adverse hematologic effects with these new vaccines remain unknown. We queried the US Centers for Disease Control Vaccine Adverse Event Reporting System (VAERS), a national surveillance database, through February 3, 2023, all reported hematologic adverse events that occurred within 42days of administration of either the Pfizer-BioNTech or Moderna Bivalent COVID-19 Booster vaccine. We included all patient ages and geographic locations and utilized 71 unique VAERS diagnostic codes pertaining to a hematologic condition as defined in the VAERS database. Fifty-five reports of hematologic events were identified (60.0% Pfizer-BioNTech, 27.3% Moderna, 7.3% Pfizer-BioNTech bivalent booster plus influenza, 5.5% Moderna bivalent booster plus influenza). The median age of patients was 66years, and 90.9% (50/55) of reports involved a description of cytopenias or thrombosis. Notably, 3 potential cases of ITP and 1 case of VITT were identified. In one of the first safety analyses of the new SARS-CoV-2 booster vaccines, we identified few adverse hematologic events (1.05 per 1,000,000 doses), most of which could not be definitively attributed to vaccination. However, three reports of possible ITP and one report of possible VITT highlight the need for continued safety monitoring of these vaccines as their use expands and new formulations are authorized.

Increased incidence of immune thrombocytopenia (ITP) in 2021 correlating with the ongoing vaccination campaign against COVID-19 in a tertiary center - A monocentric analysis.

Immune thrombocytopenia (ITP) was reported as a rare complication of COVID-19 vaccines. We conducted a retrospective single-center analysis of all ITP cases detected in 2021 and compared the quantity with the pre-vaccination years, from 2018 to 2020. In 2021, a two-fold increase in ITP cases was identified compared to previous years; 11 of 40 cases (27.5%) were considered COVID-19-vaccine related. Our study highlights an increase in ITP cases at our institution, probably related to COVID-19 vaccinations. Further studies are needed to investigate this finding globally.

Risks and outcomes of hospitalizations in patients with chronic lymphocytic leukemia admitted with immune thrombocytopenia: an analysis of the National Inpatient Sample Database.

Immune thrombocytopenia (ITP) is a known autoimmune complication of chronic lymphocytic leukemia (CLL). Currently, there is limited data regarding the risk CLL confers on hospitalization outcomes in patients admitted with ITP.The National Inpatient Sample (NIS) database was queried using the International Classification of Diseases (ICD) codes to identify hospitalizations for ITP and then subclassified the data into hospitalizations with and without CLL. A multivariate logistic regression was designed to account for patient characteristics and comorbidities. The primary outcome was all-cause mortality. Secondary outcomes included major bleeding, gastrointestinal bleeding, intracranial bleeding, and the need for platelet transfusions, intravenous immunoglobulin, and splenectomy. Among 662,171 cases of ITP between 2005 and 2019, 15,672 had concurrent CLL. CLL patients were significantly older and had more comorbidities compared to patients without CLL. Multivariate analysis revealed CLL patients with ITP had a risk of all-cause mortality (odds ratio: 1.28, 95% CI: 1.19-1.37; p < 0.01). CLL patients also had a higher risk of complications, second-line ITP treatments, blood transfusions, and bleeding, with the exception of intracranial hemorrhage. Our study suggests CLL is an independent risk factor for increased morbidity and mortality among hospitalized patients with ITP. Prospective studies are needed to determine if refractoriness to conventional treatments for ITP can account these results.

Clinical Characteristics Analysis of Immune Thrombocytopenia in Children

To analyze the clinical characteristics of immune thrombocytopenia (ITP) in children and explore the risk factors of chronic immune thrombocytopenia (CITP) . The clinical data of children newly diagnosed with ITP in the Department of Pediatrics of the First Affiliated Hospital of Wannan Medical College from January 2015 to December 2020 were retrospectively analyzed, information on age of onset, sex, blood type, platelet count at the time of initial diagnosis, mean platelet volume, lymphocyte count, platelet-related antibodies, time to normalization of platelets, and the presence of prodromal infections. The 180 cases that met the requirements were selected according to the inclusion and exclusion criteria, with at least 1 year of outpatient and telephone follow-up. The risk factors affecting the prognosis of the children were derived by univariate and muitivariate binary logistic regression analysis, and the predictive value of the risk factors was evaluated by the receiver operating characteristic curve. A total of 180 children with ITP were collected in this study, including 36 cases of chronic ITP (20%) and 144 cases of nonchronic ITP (80%). The results of univariate analysis showed that the age≥3 years, lymphocyte count≥（3.98±2.26）×109/L, and time to normalization of platelets≥（4.38±1.79）d at the time of initial diagnosis were adverse prognostic factors of CITP. The results of muitivariate analysis showed that the children with ITP aged ≥3 years (OR=3.792, 95%CI: 1.427-10.075, P=0.008), children with longer time to normalization of platelets (OR=1.295, 95%CI: 1.055-1.591, P=0.014) were more likely to develop CITP. The results of the receiver operating characteristic curve showed that the area under the curve for age prediction of CITP was 0.715, with a sensitivity of 80.6% and specificity of 61.1%; when the platelet return to normal was 4.5 days. The maximum area under the ROC curve was 0.708, with a sensitivity of 75% and specificity of 59.9%, both of which had good values for predicting CITP. Age≥3 years and longer time to normalization of platelets（≥4.5 days） are risk factors for CITP and have good value in predicting CITP, while gender, blood group, platelet count at initial diagnosis, mean platelet volume, plateletrelated antibodies, and presence of antecedent infection are not significantly correlated with CITP.

Two Cases of Immune Thrombocytopenia Associated with Tongue Hematoma

症例 1 ：81 歳，男。舌の血腫と指尖，腹部の出血斑を主訴に初診した。指尖は糖尿病の血糖値測定部位，腹部はインスリン注射部位に一致していた。血小板数は 0.5×104/μL と著明に減少していたが，PT-INR，APTT は正常範囲だった。各種臨床検査，画像所見から免疫性血小板減少症と診断した。プレドニゾロンの投与には反応せず，免疫グロブリン大量療法によって血小板数は速やかに回復した。症例 2 ：83 歳，女。舌の血腫と下腿の点状紫斑を主訴に紹介された。血小板数が 0.7×104/μL と著明に減少していたが PT-INR，APTT は正常範囲だった。各種臨床検査，画像所見から免疫性血小板減少症と診断した。プレドニゾロン 20 mg/day の投与に反応して血小板数は回復した。ピロリ菌感染も検出されたので除菌治療を行った。また，症例 2 では COVID-19 ワクチンの 3 回目接種後に発病しており，ワクチン接種との関連性についても考察した。症例 1，2 ともに舌の血腫（口腔内出血）を合併しており，血小板数減少も重篤な状態であった。過去の文献を渉猟して，口腔内出血は免疫性血小板減少症の注意すべき症状であることを強調した。

A case of Evans syndrome

Evans syndrome is an autoimmune disorder described by Robert Evans in 1951 specifying that a link exists between primary thrombocytopenic purpura and acquired haemolytic anaemia. It is a rare autoimmune disorder characterised by the simultaneous or sequential development of autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Approximately, Evans syndrome represent 5-10% of warm autoimmune haemolytic anaemia cases and 2-5% of ITP cases. It is basically a diagnosis of exclusion. We present a 50-year-old female presented with anaemia, jaundice and petechia. Patient indirect bilirubin was raised, Coombs test was positive, lactate dehydrogenase and reticulocyte count were raised. Based on the Coombs positive haemolytic anaemia and thrombocytopenia patient was diagnosed with Evans syndrome. ANA profile was positive for SS-A suggestive of underlying autoimmune disorder. Evans syndrome is difficult to diagnose and treat. Typically, corticosteroids and other immunosuppressive medications are used to manage the syndrome.

Autoimmune haemolytic anaemia and immune thrombocytopenia following SARS-CoV-2 and non-SARS-CoV-2 vaccination: 32 Years of passive surveillance data.

Autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) are two uncommon haematologic autoimmune conditions that can rarely arise secondary to vaccination. Prior studies using the US Centers for Disease Control's (CDC) Vaccine Adverse Event Reporting System (VAERS) have demonstrated this infrequency, but contemporary data as well as comparison with current information regarding SARS-CoV-2 vaccination has not been assessed. In this study, we reviewed VAERS database reports from 1990 to 2022 to characterize the incidence and clinical and laboratory findings of non-SARS-CoV-2-associated AIHA and ITP and SARS-CoV-2 vaccine-associated AIHA and ITP. We discovered a total of 863 AIHA and ITP reports following vaccination with 15 non-SARS-CoV-2 and four SARS-CoV-2 vaccines submitted to the CDC VAERS database. AIHA and ITP reporting was low for both groups, with a large proportion excluded due to a lack of clinical details. ITP was reported the most frequently in both groups and was significantly more common with measles-mumps-rubella (MMR) vaccination (p < 0.001) in the non-SARS-CoV-2 group. AIHA and ITP cases were higher in the SARS-CoV-2 vaccine group, though ultimately still very infrequent. Autoimmune haematologic disease is vanishingly rare after immunization and rates are lower than in the general population according to passive reporting.

Aetiology and Outcomes of Thrombocytopenia in Pregnancy: A Cross-Sectional Study in a University Hospital, India

Background: Thrombocytopenia (TCP) is the second most common haematological finding in pregnancy next to anaemia. It carries a risk for both the mother and the fetus, associated with substantial maternal or neonatal morbidity and mortality. However, a specific therapy, if instituted promptly, improves the outcome for affected patients and their offspring. In patients in India, TCP during pregnancy is an underexplored condition. Objectives: To assess the aetiology of TCP in pregnancy and to assess the maternal outcomes of TCP in pregnancy. Methodology: The authors included a total of 133 patients in their third trimester (&gt;32 weeks), with a platelet count &lt;149,000 /mm3, admitted to the authors’ institution from 1st January 2021 to 31st December 2021. Patient-related data such as menstrual and obstetric history, presenting complaints, obstetric examination, and basic investigations were collected in a pre-designed, pre-tested proforma. All cases were followed until delivery to record any maternal complications, or any other morbidities. The data were analysed using SPSS (International Business Machines Corporation, Armonk, New York, USA) software. χ2 test was used to compare the proportions between the groups. p&lt;0.05 was considered significant. Results: Overall, 64.7% of patients were in the 18–25 years age group and 49.6% of patients were primigravida. Furthermore, 60.9% of patients were diagnosed to have mild TCP, 32.3% had moderate TCP, and only 6.8% patients had severe TCP. The majority (75.2%) of cases were of gestational TCP. In total, 15.8% of cases had pregnancy-induced hypertension (PIH); 3.0% had dengue; 2.3% were COVID-19 positive; 1.5% were diagnosed with haemolysis, elevated liver enzymes, and low platelets syndrome; 1.5% had immune TCP; and only one patient had leptospirosis. Four percent of cases had gestational TCP, 9.5% had PIH, one patient (25.0%) had dengue, and both cases of immune TCP had severe TCP. Twenty-eight percent of gestational TCP cases; 47.6% of PIH cases, both cases of haemolysis, elevated liver enzymes, and low platelets syndrome; 50.0% of dengue cases; and one COVID-19 positive case (33.0%) had moderate TCP. Finally, 6.25% of patients who underwent lower segment caesarean section had severe TCP, 6.00% of patients who underwent vaginal delivery had severe TCP, and out of two patients who had a spontaneous abortion, one (50.00%) had severe TCP at the time of admission. The association was significant (p&lt;0.05). Conclusion: TCP is a crucial condition among pregnant patients. Mild TCP is a common type. Correct aetiological diagnosis, and promptly administered adequate and specific therapy are, therefore, essential to significantly improve the outcomes of pregnant patients and their offspring.

Cost-Effectiveness of a Quality Improvement Initiative for the Clinical Management of Children with Newly Diagnosed Immune Thrombocytopenia

To assess the cost-effectiveness of an evidence-informed institutional protocol for physicians that encouraged management of children with newly diagnosed immune thrombocytopenia (ITP) with observation over active therapy, where appropriate. We conducted a probabilistic cost-effectiveness analysis from an institutional perspective using a decision tree with a 1year time horizon. Patient-level data were retrospectively ascertained for children diagnosed in pre-protocol (2007-2009) and post-protocol (2013-2018) time periods. ITP resolution was defined as achieving a sustained platelet count of >100×103/μL at 9-12months after diagnosis. Outpatient care and inpatient costs were obtained from the institution and provincial sources. Intervention costs accounted for quality improvement initiative preparation and staff physician training. Incremental costs, incremental effects, and CIs were calculated from 10 000 model iterations. Forty-eight patients were followed for 1year in the pre-protocol period and 84 in the post-protocol period. After protocol implementation, an average cost savings per child managed of $2055 (95% CI: $656, $3890) Canadian Dollars was observed, as was a higher proportion of resolved ITP cases. The implementation strategy remained less costly and more effective in 99.7% of model iterations. Implementation of an evidence-informed institutional protocol to guide physicians toward increased uptake of observation over active therapy when managing children with newly diagnosed ITP resulted in significant cost savings on a per case basis, even after accounting for training-related costs. Though the long-term cost implications regarding the sustainability of the intervention are not yet known, it is anticipated that continued institutional savings could occur.