EJHaem最新文献

{"title":"Prophylactic versus on-demand transfusion in pregnant women with sickle cell disease: A systematic review and meta-analysis of randomised controlled trials","authors":"Denise Menezes Brunetta,&nbsp;Evangelia Vlachodimitropoulou,&nbsp;Nita Prasannan,&nbsp;Paul T. Seed,&nbsp;Eugene Oteng-Ntim","doi":"10.1002/jha2.1086","DOIUrl":"https://doi.org/10.1002/jha2.1086","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Sickle cell disease (SCD) poses significant risks during pregnancy. Transfusions are the only recommended treatment, but there is no strong evidence of its efficacy. The aim of this study was to evaluate prophylactic transfusion on pregnancy outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We performed a systematic review and meta-analysis (PROSPERO-CRD42024510511), using MEDLINE, EMBASE, Cochrane, Web of Science, and Maternity and Infant Care. No date or language restrictions were applied. Inclusion criteria comprised randomised-controlled trials (RCTs) involving SCD pregnancy, comparing maternal and foetal outcomes for prophylactic versus on-demand transfusions. Two independent reviewers performed screening, selection, and data extraction, following PRISMA. Two authors independently assessed certainty and risk-of-bias. Data were pooled using random-effects model. Primary outcomes included mortality, vaso-occlusive crisis (VOC), acute chest syndrome, venous thromboembolism and preterm delivery. The measure of the effect was the unadjusted odds ratio (OR), calculated from numbers of events.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Ninety-one studies were identified and two RCTs (106 patients) were included, with uncertain and low risk of bias. Prophylactic transfusions reduced VOC, OR of 0.197 (95% CI 0.08–0.49). However, due to the small number of patients, this meta-analysis was underpowered to evaluate other outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>A larger RCT is needed to comprehensively assess the impact of prophylactic transfusion in SCD pregnancy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1086","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143865850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immune Thrombocytopenia in an Adult With X-linked Agammaglobulinemia: A Case Report","authors":"Takeaki Matsunaga,&nbsp;Ken Naganuma,&nbsp;Noriko Tanabe,&nbsp;Yoshiko Mori,&nbsp;Marino Nagata,&nbsp;Shuji Momose,&nbsp;Yasushi Kubota","doi":"10.1002/jha2.1101","DOIUrl":"https://doi.org/10.1002/jha2.1101","url":null,"abstract":"<p>In patients with X-linked agammaglobulinemia (XLA), serum immunoglobulins are almost completely lacking. The prevalence of autoimmune diseases is low in XLA compared with other primary immunodeficiency diseases because antibodies are absent in XLA. Immune thrombocytopenia (ITP) is considered an antibody-mediated disease characterized by increased platelet destruction, and adult-onset ITP in XLA has not been reported in detail. The case of a 29-year-old Japanese man with XLA and ITP is described. The patient was treated with prednisolone and intravenous immunoglobulins, resulting in rapid improvement of thrombocytopenia. Clinicians should consider co-existing ITP when progressive thrombocytopenia is observed in a patient with XLA.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1101","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143865851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Iron Deficiency Anemia and Ischemic Stroke in Young Adults","authors":"Jahnavi Gollamudi,&nbsp;Sadeer Al-Kindi,&nbsp;Lalitha Nayak","doi":"10.1002/jha2.70008","DOIUrl":"https://doi.org/10.1002/jha2.70008","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Iron deficiency anemia (IDA) is the most common cause of anemia and is linked to higher arterial stroke risk in children, but its effect in adults remains unclear.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We performed a retrospective study examining the prevalence of IDA in young adults with and without ischemic stroke.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We found that the prevalence of IDA was five times higher in patients with ischemic stroke than those without stroke (5% vs. 1.05%).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>IDA independently increased the odds of an ischemic stroke by 39% (<i>p</i> &lt; 0.001). Given the global burden of IDA, effective screening strategies are necessary.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70008","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143861796","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Concurrent Bone Marrow Acute Undifferentiated Leukemia and Mediastinal T-Lymphoblastic Lymphoma With Identical SET::NUP214 Fusion and PHF6 and EZH2 Mutations","authors":"Qing Wei,&nbsp;Fatima Z. Jelloul,&nbsp;Sa A. Wang,&nbsp;Guilin Tang,&nbsp;Shaoying Li,&nbsp;Pei Lin,&nbsp;Sanam Loghavi,&nbsp;Huan-You Wang,&nbsp;L. Jeffrey Medeiros,&nbsp;Jie Xu","doi":"10.1002/jha2.70034","DOIUrl":"https://doi.org/10.1002/jha2.70034","url":null,"abstract":"<p>Acute undifferentiated leukemia (AUL) is a rare hematologic malignancy lacking lineage-specific markers. Concurrent, clonally related AUL and T-lymphoblastic lymphoma (T-LBL) has not been reported previously. Here we describe a patient who was diagnosed with AUL in the bone marrow and T-LBL in the mediastinum after a thorough immunophenotyping by flow cytometry and immunohistochemistry. Despite their immunophenotypic differences, the AUL and T-LBL showed identical genetic alterations: <i>SET::NUP214</i> fusion, <i>PHF6</i>, and <i>EZH2</i> mutations. The patient achieved and remained in complete remission after chemotherapy and stem cell transplantation. This case underscores the value of comprehensive immunophenotyping and genetic analysis in rare hematologic malignancies.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70034","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143856900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Blasts With Cup-Like Nuclear Morphology: When the First Impression Could Be Misleading","authors":"Délaissée Nyamessameye,&nbsp;Marie Vercruyssen,&nbsp;Danai Poutakidou,&nbsp;Pierre Heimann,&nbsp;Marco Fangazio,&nbsp;Anne-laure Trepant,&nbsp;Hussein Farhat","doi":"10.1002/jha2.70022","DOIUrl":"https://doi.org/10.1002/jha2.70022","url":null,"abstract":"&lt;p&gt;A 53-year-old man was referred to the hematology department for evaluation of newly diagnosed pancytopenia. He was undergoing maintenance therapy with lenalidomide following autologous stem cell transplantation (ASCT) for multiple myeloma (MM), diagnosed 4 years earlier.&lt;/p&gt;&lt;p&gt;Bone marrow aspiration revealed 90% blasts, some with cup-like nuclear morphology, a feature classically associated with certain acute myeloid leukemia (AML) harboring &lt;i&gt;NPM1&lt;/i&gt; and/or &lt;i&gt;FLT3&lt;/i&gt; mutations (Figure 1A, May–Grunwald–Giemsa stain, original magnification ×1000). However, immunophenotyping demonstrated a B-cell precursor acute lymphoblastic leukemia (B-ALL) phenotype. The blasts were CD19+/CD10partial/CD20−/CD22+/CD33+/CD34+/cytoplasmic CD79a+/TdT+/cytoplasmic IgM+/MPO− (Figure 1C). No monoclonal plasma cells were detected, and kappa/lambda light chains were assessed only by flow cytometry, where they were detected in a few polyclonal lymphocytes but not on the blasts.&lt;/p&gt;&lt;p&gt;Molecular analyses identified a complete deletion of the &lt;i&gt;CDKN2A-CDKN2B&lt;/i&gt; locus, with no mutations detected in &lt;i&gt;NPM1&lt;/i&gt; or &lt;i&gt;FLT3&lt;/i&gt;. Cytogenetic testing revealed a normal karyotype, while the patient's initial MM was characterized by hypotriploidy with gains of chromosomes 3, 5, 6, 7, 9, 11, 15, 17, 19, and 20.&lt;/p&gt;&lt;p&gt;Immunohistochemistry (IHC) demonstrated diffuse cytoplasmic IgM positivity (Figure 1B, IHC, original magnification ×1000). This finding could raise a differential diagnosis with Dutcher bodies, which are cytoplasmic immunoglobulin inclusions typically found in plasma cells and mature B cells. However, the diffuse IgM staining pattern observed in our case does not fit with Dutcher bodies, which appear as well-defined, localized inclusions that stain strongly positive. Instead, the perinuclear clearing observed could suggest unstained Golgi zones.&lt;/p&gt;&lt;p&gt;The patient achieved complete remission after 2 months of B-ALL-directed chemotherapy, followed by allogeneic bone marrow transplantation.&lt;/p&gt;&lt;p&gt;Cup-like nuclear morphology has been well-described in AML, particularly in cases with &lt;i&gt;NPM1&lt;/i&gt; and/or &lt;i&gt;FLT3&lt;/i&gt; mutations, but has been rarely reported in B-ALL. Few cases in the literature describe B-ALL with similar nuclear features, including cases associated with &lt;i&gt;IGH::DUX4&lt;/i&gt; fusion, &lt;i&gt;BCR-ABL1&lt;/i&gt; translocation, or &lt;i&gt;t&lt;/i&gt;(4;11) rearrangement. Notably, our case lacked these alterations, suggesting that cup-like nuclear morphology in B-ALL may not be restricted to a single molecular mechanism.&lt;/p&gt;&lt;p&gt;This case also highlights the role of genetic instability in secondary hematologic malignancies. While no shared cytogenetic abnormalities were identified between the initial multiple myeloma and the secondary leukemia, the presence of hypotriploidy in the MM clone and the occurrence of a &lt;i&gt;CDKN2A-CDKN2B&lt;/i&gt; deletion in the B-ALL clone suggest a clonal evolution process.&lt;/p&gt;&lt;p&gt;Lenalidomide exposure and prior ASCT have been associated with an increased risk of se","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70022","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143846180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Massive Intramuscular Haematoma Due to Acquired Factor V Deficiency","authors":"Lara Budwig,&nbsp;Varvara Bashkirova,&nbsp;Shital Shah,&nbsp;David Maudgil,&nbsp;Dalia Khan,&nbsp;John Willan","doi":"10.1002/jha2.70030","DOIUrl":"https://doi.org/10.1002/jha2.70030","url":null,"abstract":"&lt;p&gt;A 95-year-old retired bus driver presented with a 4-day history of pain and swelling in the left thigh. There was no history of trauma, infection or medication changes. He had a history of ischaemic stroke, hypertension and diabetes mellitus, he did not drink alcohol in excess, and was previously living independently. His regular medications included clopidogrel, which was stopped on admission. Laboratory tests showed haemoglobin (Hb) of 63 g/L [130–180], prothrombin time (PT) of 68.1s [10.5–14.7] and activated partial thromboplastin time (aPTT) of 209 s [26.4–35.9] with 95% correction on immediate 50/50 mixing studies. Clotting tests had been normal when last tested in 2009. There was no evidence of hepatic dysfunction. Computed tomography of the left thigh (Figure 1 left panel) showed an extensive intramuscular haematoma measuring 28 cm × 10 cm × 10 cm with multiple areas of blushing; there was no evidence of malignancy on full body imaging. Initial management was with fresh frozen plasma (FFP) for a presumed consumptive coagulopathy, along with vitamin K, red cell transfusion and tranexamic acid, however, only minimal improvement in coagulation assays was noted (Figure 1 right panel).&lt;/p&gt;&lt;p&gt;Factor V level (FV), taken after FFP replacement had been initiated, was 1 IU/dL [50–150] with a positive FV inhibitor screen at 0.8 Bethesda Units. Fibrinogen and other clotting factor levels were normal. 1 mg/kg prednisolone was started as first line immunosuppression on day 10.&lt;/p&gt;&lt;p&gt;Despite these interventions, Hb dropped to 55 g/L on day 12 with concern of re-bleeding into the haematoma. Regular platelet transfusions were initiated (since their alpha-granules are rich in FV, potentially delivering this factor to the site of primary haemostasis), and 0.4 g/kg doses of intravenous immunoglobulin (IVIg) were given on days 14 and 17. Unfortunately the patient tested positive for SARS-CoV-2 on day 15 and clinically deteriorated with renal injury, fluid overload and worsening respiratory infection despite antimicrobial therapy. Plasma exchange was proposed to allow time for the immunosuppression to take effect, but the patient declined this. On day 21 the patient became septic with haemodynamic instability, and passed away the following day.&lt;/p&gt;&lt;p&gt;Acquired FV inhibitors are very rare (estimated incidence of 0.02–0.09 per million persons per year). Triggers include bovine thrombin, antibiotics, infection, malignancies and autoimmune conditions, however up to 30% of cases are idiopathic as in this case. FV levels, inhibitor titre and degree of coagulation profile derangement correlate poorly with likelihood and severity of bleeding, therefore, careful clinical assessment is crucial.&lt;/p&gt;&lt;p&gt;The paper was drafted by Lara Budwig, Varvara Bashkirova, John Willan. All authors reviewed, edited, and approved the manuscript for publication. The care of the patient was delivered by all authors, with interpretation of radiology images by DM.&lt;/p&gt;&lt;p&gt;The authors declare ","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70030","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143826822","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autoimmune Haemolytic Anaemia in Patients With Chronic Liver Disease: Case Series","authors":"Dorien Pint,&nbsp;Abid Suddle,&nbsp;M. Mansour Ceesay","doi":"10.1002/jha2.70035","DOIUrl":"https://doi.org/10.1002/jha2.70035","url":null,"abstract":"&lt;p&gt;Chronic anaemia frequently accompanies chronic liver disease (CLD), contributing to increased morbidity and mortality rates [&lt;span&gt;1&lt;/span&gt;]. In up to 53% of cases, the aetiology of anaemia in CLD remains unknown [&lt;span&gt;2&lt;/span&gt;]. Autoimmune haemolytic anaemia (AIHA) is characterised by the destruction of red blood cells by circulating autoantibodies directed against erythrocytes. AIHA is a rare autoimmune disorder affecting approximately one to three per 100,000 annually [&lt;span&gt;3&lt;/span&gt;]. AIHA has a female predisposition with a female-to-male ratio of approximately 2:1 to 3:1 [&lt;span&gt;4&lt;/span&gt;]. The diagnosis of AIHA has additional challenges in CLD, as all these laboratory values can be distorted due to the CLD. For instance, haptoglobin is low in CLD due to poor synthetic function, and similarly, LDH is often raised without haemolysis [&lt;span&gt;5&lt;/span&gt;]. Autoimmune liver and bile duct diseases such as autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cholangitis (PBC) often coexist with other autoimmune disorders, though AIHA is a rare comorbidity.&lt;/p&gt;&lt;p&gt;We conducted a retrospective study from 2002 to 2022 investigating patients with concomitant CLD and AIHA managed in King's College Hospital Institute of Liver Studies and the Department of Haematological Medicine. The patients were identified using search criteria for various causes of CLD combined with AIHA diagnosis. The diagnosis of AIHA was based on laboratory parameters. The review aimed to evaluate the relationship between AIHA and CLD as well as review the diagnostic and therapeutic challenges. Due to the retrospective nature of this study, we were unable to determine the exact number of patients with CLD seen in our institution during the study period. As a result, we could not establish a denominator for AIHA prevalence in this population. However, our objective was not to perform an epidemiological assessment but rather to describe the clinical complexities in diagnosing and managing these rare cases.&lt;/p&gt;&lt;p&gt;A total of 10 patients were included in this study (Table 1). The median age for the diagnosis of chronic liver disease was 34 years (range 6–72), and the median age of AIHA diagnosis was 43 (19–72 years). The median interval between CLD diagnosis and onset of AIHA was 6 (range 0–17) years. There was female predominance with 70% being female. All patients had classical features of haemolysis with an elevated unconjugated bilirubin (mean 88.5 µmol/L), increased LDH (mean 496 U/L), reduced haptoglobin, and positive direct antiglobulin test (DAT). The most common underlying liver disease was AIH in four patients. Seven patients had cirrhosis, and five received an orthotopic liver transplantation (OLT). Among the five transplanted patients, three developed AIHA after their liver transplant. Indications for liver transplantation were AIH in two patients, and primary sclerosing cholangitis (PSC) and alcohol-related liver disease (ArLD) both in one pat","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70035","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143818347","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploring Cryoglobulinemia's Clinical Odyssey: A Case Series","authors":"Shivangini Duggal,&nbsp;Lakshmi Vaishnavi Prasanna Kattamuri,&nbsp;Michel Toutoungy,&nbsp;Eder Luna Ceron,&nbsp;Madhumita Rondla,&nbsp;Angelica Lehker","doi":"10.1002/jha2.70029","DOIUrl":"https://doi.org/10.1002/jha2.70029","url":null,"abstract":"<p>Cryoglobulinemia (CG) encompasses disorders in which immunoglobulins precipitate at low temperatures. According to Brouet's classification, Type I CG is linked to plasma cell disorders, while mixed CG (Types II and III) is associated with autoimmune conditions, infections (notably hepatitis C virus [HCV]), and lymphoproliferative disorders. Each type presents distinct symptoms, with Type I often causing severe vasomotor symptoms and Types II and III involving systemic features like purpura, Raynaud's phenomenon, and renal involvement. This case series presents three CG cases, illustrating diverse etiologies and presentations. Case 1 discusses CG in metastatic colon cancer with <i>Staphylococcus aureus</i> bacteremia, highlighting infection-triggered CG. Case 2 covers HCV-related mixed CG, emphasizing antiviral therapy's role. Case 3 describes a CG flare after rituximab therapy, managed with steroids. These cases emphasize a multidisciplinary approach and individualized management to address CG's complexity and improve patient outcomes.</p><p><b>Clinical Trial Registration</b>: The authors have confirmed clinical trial registration is not needed for this submission.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70029","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143749853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Effect of COVID-19 on Platelet Counts in Persistent and Chronic Adult ITP Patients: A Real-World Study in China","authors":"Yujiao Zhang,&nbsp;Lei Yang,&nbsp;Zhongping Xu,&nbsp;Xin Zhou","doi":"10.1002/jha2.70025","DOIUrl":"https://doi.org/10.1002/jha2.70025","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>While coronavirus disease 2019 (COVID-19)-associated thrombocytopenia is well-documented, its effects on immune thrombocytopenia (ITP) patients remain unclear. This study aimed to investigate the impact of COVID-19 infection on platelet (PLT) dynamics in chronic ITP patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This retrospective study analyzed 21 persistent and chronic ITP patients before and after mild COVID-19 infection during China's December 2022 reopening, comparing platelet parameter changes with a focus on clinical characteristics of thrombopoietin receptor agonist (TPO-RA) treated patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>TPO-RA treated patients demonstrated transient platelet surges peaking at 1 week postinfection, returning to baseline within 2–3 weeks, contrasting sharply with thrombocytopenia patterns in non-ITP populations. This suggests synergistic effects between virus-induced inflammatory cytokines and TPO-RA may drive transient megakaryopoiesis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>These findings underscore infection-related PLT fluctuations in ITP, necessitating monitoring for thrombotic and bleeding risks and TPO-RA dose optimization during infections.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Trial Registration</h3>\u0000 \u0000 <p>The authors have confirmed clinical trial registration is not needed for this submission</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70025","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143735436","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lymphocytic Variant Hypereosinophilic Syndrome: Case Series From a Tertiary Referral Center in Canada","authors":"Xiu Qing Wang,&nbsp;Kevin Shopsowitz,&nbsp;Jack Lofroth,&nbsp;Xuehai Wang,&nbsp;Erica Peterson,&nbsp;Andrew P. Weng,&nbsp;Luke Y. C. Chen","doi":"10.1002/jha2.1109","DOIUrl":"https://doi.org/10.1002/jha2.1109","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Lymphocytic variant hypereosinophilic syndrome (L-HES) is a rare disorder characterized by persistent eosinophilia driven by aberrant T-cell populations. Diagnosis remains challenging due to the lack of standardized diagnostic criteria.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We retrospectively analyzed 18 patients diagnosed with L-HES between 2016 and 2023. Comprehensive flow cytometry was performed on peripheral blood samples.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Nine patients demonstrated the classic sCD3⁻CD4⁺CD5⁺CD2⁺CD45RO⁺CD45RA⁻ immunophenotype, ranging from 0.6% to 70% of total lymphocytes. Two patients showed variant sCD3⁻CD4⁺ phenotypes, five had expanded (&gt; 10%) sCD3⁺CD4⁺CD7⁻ T-cells, and two displayed aberrant CD8⁺ T-LGL populations. Clonality was established in all patients with nonclassic phenotypes by molecular TCR testing or based on uniform TRBC1. We assessed a serial gating strategy to quantify the classic L-HES phenotype and found this to be highly sensitive and specific with an estimated limit of detection of 0.06% of lymphocytes. Using this strategy, we identified decreased but detectable abnormal T-cells in all classic phenotype patients reassessed posttreatment, down to as low as 0.3% of lymphocytes. The identification of T-LGL phenotypes with eosinophilia is a novel finding.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Our study highlights the diverse immunophenotypic spectrum of L-HES, emphasizing the importance of comprehensive flow cytometry analysis for accurate diagnosis.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1109","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143735435","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}