EJHaem最新文献

{"title":"Factors related to mean corpuscular volume in HFE p.C282Y homozygotes","authors":"James C. Barton,&nbsp;J. Clayborn Barton,&nbsp;Ronald T. Acton","doi":"10.1002/jha2.1063","DOIUrl":"10.1002/jha2.1063","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>The aim of this study was to define the relationships between factors other than transferrin saturation (TS) to mean corpuscular volume (MCV) and macrocytosis (MCV &gt; 100 fL) in <i>HFE</i> p.C282Y (rs1800562) homozygotes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We studied white post-screening participants with p.C282Y homozygosity who did not have anemia, report cirrhosis or pregnancy, or use medications that increase MCV. We analyzed relations of MCV and macrocytosis with age, sex, diabetes reports, daily alcohol consumption, swollen or tender 2nd/3rd metacarpophalangeal (MCP) joints, TS, and serum ferritin (SF).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>There were 257 participants (110 men and 147 women). Median alcohol consumption, median TS, median SF, and macrocytosis prevalence were significantly greater in men. Relative risk of macrocytosis in men was 2.81. In men and women, there were significant positive Pearson's correlations of MCV versus age and Spearman's correlations of MCV versus alcohol consumption and TS. Mean MCV and macrocytosis prevalence were significantly greater in participants with than without swollen or tender 2nd/3rd MCP joints. Linear regression on MCV revealed positive associations: age (<i>p</i> &lt; 0.0001), alcohol consumption (<i>p</i> = 0.0007), and TS (<i>p</i> &lt; 0.0001). Logistic regression on macrocytosis revealed an odds ratio for age of 1.04 (95% confidence interval: 1.00, 1.07).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>MCV in <i>HFE</i> p.C282Y homozygotes is positively related to age, daily alcohol consumption, TS, and swollen or tender 2nd/3rd MCP joints.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756985/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048814","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":"Masked polycythemia vera in a patient 5 years after gastric bypass surgery: A diagnostic pitfall","authors":"Daniel Rivera,&nbsp;Carlos E. Bueso-Ramos,&nbsp;L Jeffrey Medeiros,&nbsp;Beenu Thakral","doi":"10.1002/jha2.1068","DOIUrl":"10.1002/jha2.1068","url":null,"abstract":"&lt;p&gt;A 66-year-old woman presented with a fever and symptoms of urinary tract infection. A complete blood count showed: white blood cells (WBC) 26.2 × 10&lt;sup&gt;9&lt;/sup&gt;/L(normal, 4–11 × 10&lt;sup&gt;9&lt;/sup&gt;/L), red blood cells (RBC) 7.2 × 10&lt;sup&gt;12&lt;/sup&gt;/L(normal 3.99–5.46 × 10&lt;sup&gt;12&lt;/sup&gt;/L), hemoglobin 12.4 g/dL(normal, 12.2–15.3 g/dL), hematocrit 45.3%(normal 36.4%–46.8%), mean corpuscular volume 63 fL(normal, 82–99 fL) and platelets 631 × 10&lt;sup&gt;9&lt;/sup&gt;/L(normal, 160–397 × 10&lt;sup&gt;9&lt;/sup&gt;/L). Serum iron was 15 µg/dL and ferritin was 10 ng/mL. A review of the peripheral blood smear (panel A) showed microcytic RBCs and pencil cells on the peripheral blood smear suggestive of iron deficiency. However, the high RBC count did not fit with iron deficiency, and thus bone marrow evaluation was performed. The biopsy specimen was hypercellular (∼90%) with panmyelosis and pleomorphic megakaryocytic hyperplasia without dysplasia or increased blasts (panels B–D). Absent storage iron was seen on iron stain performed on an aspirate smear. Further work-up showed that serum erythropoietin (EPO) was low and a detailed review of the medical history revealed the patient underwent gastric bypass surgery ∼5 years ago. Ultrasound abdomen detected mild splenomegaly (15.5 cm). Next-generation sequencing study showed &lt;i&gt;JAK2&lt;/i&gt; p.V617F(VAF:83%) mutation and conventional cytogenetics showed a normal diploid female karyotype. These results confirmed the diagnosis of “masked” polycythemia vera (PV) with concurrent iron deficiency likely due to gastric bypass (Figure 1).&lt;/p&gt;&lt;p&gt;According to the current World Health Organization classification and the International Consensus Classification, the diagnosis of PV requires three major criteria or two major with one minor criterion [&lt;span&gt;1, 2&lt;/span&gt;]. The three major criteria are 1) An elevated hemoglobin (≥16.5 g/dL in men and ≥16.0 g/dL in women) or elevated hematocrit (≥49% in men and ≥48% in women); 2) Bone marrow biopsy specimen with age-adjusted hypercellularity with panmyelosis and pleomorphic megakaryocytic proliferation, and 3) Presence of &lt;i&gt;JAK2&lt;/i&gt; p.V617F or &lt;i&gt;JAK2&lt;/i&gt; exon 12 mutation; the minor criterion is a subnormal EPO level [&lt;span&gt;1, 2&lt;/span&gt;]. Gastric bypass surgery compromises iron absorption by bypassing the portion of the small intestine where iron is absorbed, thus decreasing gastric acid production as a feedback loop. Furthermore, calcium and zinc may compete with iron for absorption and cause iron deficiency. Normal hemoglobin and hematocrit levels in the setting of chronic iron deficiency create a diagnostic pitfall when trying to meet diagnostic criteria for PV [&lt;span&gt;3, 4&lt;/span&gt;]. This scenario is a diagnostic pitfall and can delay the diagnosis of PV or even result in misdiagnosis as essential thrombocythemia or pre-fibrotic myelofibrosis. Awareness and knowledge of gastric bypass as an etiology of iron deficiency can help in the appropriate diagnosis, management, and risk stratification in PV patients. To","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756979/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048891","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":"Association of the nutritional risk index recorded prior to allogeneic hematopoietic cell transplantation with the clinical prognosis in children","authors":"Hitomi Yonesu,&nbsp;Satoru Hamada,&nbsp;Hideki Sakiyama,&nbsp;Shinobu Kiyuna,&nbsp;Tokiko Oshiro,&nbsp;Nobuyuki Hyakuna,&nbsp;Koichi Nakanishi","doi":"10.1002/jha2.1054","DOIUrl":"10.1002/jha2.1054","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>The nutritional risk index (NRI), calculated using serum albumin levels and body weight ratio is a known prognostic factor in adult hematopoietic cell transplantation (HCT). However, its usefulness in pediatric HCT settings remains unclear.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In a retrospective study, we examined pre-transplant NRI impact on outcomes in 82 pediatric patients undergoing allogeneic HCT.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The 2-year non-relapse mortality (NRM) rate was 7.94% (95% confidence interval [CI], 3.05%–19.8%) and 30.8% (95% CI, 16.7%–52.2%) in the high and low NRI groups, respectively (<i>p</i> = 0.0037).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>We found that poor nutritional status prior to pediatric HCT led to a worse prognosis, including increased NRM.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756969/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048789","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":"Myeloproliferative neoplasm harboring both monosomy 7 and an ALK/ROS1 fusion gene: Proposal for a new disease entity","authors":"Hideki Yoshida,&nbsp;Shinya Osone,&nbsp;Madoka Konishi,&nbsp;Seiji Tanaka,&nbsp;Tohru Inaba,&nbsp;Toshihiko Imamura,&nbsp;Tomoko Iehara","doi":"10.1002/jha2.1071","DOIUrl":"10.1002/jha2.1071","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>We present a case of a 9-year-old girl diagnosed with a myeloproliferative neoplasm (MPN) harboring both monosomy 7 and an ALK/ROS1 fusion gene.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case presentation</h3>\u0000 \u0000 <p>The neoplasm was resistant to conventional AML chemotherapy and required hematopoietic cell transplantation (HCT) to achieve remission.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>MPNs with monosomy 7 and ALK/ROS1 fusions occur in a wide age range of children and adults, and require HCT for long-term remission. Furthermore, these cases can be responsive to ALK inhibitors.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This report underscores the potential need to reclassify such MPNs as a distinct entity, which has unique therapeutic implications.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756977/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048941","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":"Clinical, immunophenotypic, and genomic findings of acute myeloid leukemia with RAM immunophenotype: Comparison with other CD56-positive acute leukemias","authors":"Hanan Hamdan,&nbsp;Yen-Chun Liu,&nbsp;Sa A. Wang,&nbsp;Jacob Bledsoe,&nbsp;Karen M. Chisholm,&nbsp;Alexa Siddon,&nbsp;Robert Ohgami,&nbsp;Tracy I. George,&nbsp;Jason Kurzer,&nbsp;Robert P. Hasserjian,&nbsp;Daniel A. Arber,&nbsp;Adam Bagg,&nbsp;Kathryn Foucar,&nbsp;Elizabeth Margolskee,&nbsp;Dorottya Laczko,&nbsp;Weina Chen,&nbsp;Franklin Fuda,&nbsp;Nidhi Aggarwal,&nbsp;Olga K. Weinberg","doi":"10.1002/jha2.1052","DOIUrl":"10.1002/jha2.1052","url":null,"abstract":"&lt;div&gt;\u0000 \u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Background&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Acute myeloid leukemia (AML) with RAM immunophenotype is a newly recognized high-risk AML immunophenotypic subcategory characterized by blasts with bright expression of CD56 and weak to absent expression of CD45, HLA-DR, and CD38, as first described by the Children's Oncology Group (COG). The relationship between AML-RAM and other CD56-positive acute leukemias is unclear. The goal of this study is to characterize the clinicopathological characteristics of AML with RAM phenotype and compare them with other CD56 co-expressing acute leukemias.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Methods&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;From a multi-institutional search, we identified a total of 160 CD56+ acute leukemia cases, including AML-RAM (&lt;i&gt;n&lt;/i&gt; = 28), CD56+ acute undifferentiated leukemia (AUL) (&lt;i&gt;n&lt;/i&gt; = 11), CD56+ T-lymphoblastic leukemia (&lt;i&gt;n&lt;/i&gt; = 39), and CD56+ AML (&lt;i&gt;n&lt;/i&gt; = 81). We compared the clinical and pathologic findings of these groups.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Results&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;AML-RAM patients were significantly younger and presented with significantly higher platelet and white blood cell counts and bone marrow (BM) blast percentages when compared to AUL (&lt;i&gt;p&lt;/i&gt; &gt; 0.05) and had higher median BM blast percentages than T-ALL and CD56+ AML groups (both &lt;i&gt;p&lt;/i&gt; &lt; 0.05). Flow cytometry showed significantly brighter expression of CD56 on blasts as compared to other CD56+ AML cases, partial CD34 expression compared to AUL, and AML, weak-to-absent CD38 expression compared to all groups, and absent HLA-DR and terminal deoxynucleotidyl transferase as compared to AUL and T-ALL (all &lt;i&gt;p&lt;/i&gt; &lt; 0.05). The frequency of abnormal karyotypes was significantly higher among RAM when compared to all groups (&lt;i&gt;p&lt;/i&gt; &lt; 0.05). Next-generation sequencing profiles differed among the leukemia groups, with significant enrichment of &lt;i&gt;CBFA2T3::GLIS2&lt;/i&gt; fusions (&lt;i&gt;p&lt;/i&gt; &lt; 0.05) and &lt;i&gt;TP53&lt;/i&gt; mutations (&lt;i&gt;p&lt;/i&gt; &lt; 0.05) in RAM cases compared to other AML control groups, and &lt;i&gt;U2AF1&lt;/i&gt; (&lt;i&gt;p&lt;/i&gt; &lt; 0.05), serine and arginine-rich splicing factor 2 (&lt;i&gt;p&lt;/i&gt; &lt; 0.05), and BCL6 co-repressor (&lt;i&gt;p&lt;/i&gt; &lt; 0.05) mutations compared to AUL. Clinical outcome analysis demonstrated significantly lower 3-year overall survival of the RAM subgroup (36 months) compared to control groups (&lt;i&gt;p&lt;/i&gt; = 0.002).&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Conclusion&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;We find that AML with RAM phenotype occurs primarily in younger ages, with distinct clinicopathological, immunophenotypic, and mutational presentations, and worse prognosis. This diagnosi","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756974/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048796","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":"Characteristics of relapsed/refractory diffuse large B-cell lymphoma patients with durable responses to maveropepimut-S, pembrolizumab, and cyclophosphamide: Long-term follow-up from the SPiReL trial","authors":"Arjun Pandey,&nbsp;Kim Roos,&nbsp;Yidi Jiang,&nbsp;Kathryn Mangoff,&nbsp;Gail Klein,&nbsp;Nick Forward,&nbsp;Douglas Stewart,&nbsp;Pierre Laneuville,&nbsp;Isabelle Bence-Bruckler,&nbsp;Joy Mangel,&nbsp;George Tomlinson,&nbsp;Neil L. Berinstein","doi":"10.1002/jha2.1062","DOIUrl":"10.1002/jha2.1062","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Patients with relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL) have an unmet medical need. The objective of this trial was to assess the efficacy and toxicities of a novel triple immunotherapy regimen—pembrolizumab, low-dose cyclophosphamide, and maveropepimut-S (MVP-S). This regimen was designed to activate tumor-specific T cells by targeting the tumor-associated antigen survivin with MVP-S and reducing two important T cell inhibitory pathways: T cell exhaustion and regulatory T cells with pembrolizumab and metronomic cyclophosphamide, respectively.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a single-arm Phase II clinical trial in 25 participants with R/R DLBCL-SPiReL trial (NCT03349450).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The median overall survival was 10.1 months and a third of participants survived over 2 years. Enhanced long-term survival was associated with favorable clinical characteristics and enhanced immune reactivity, as assessed by ELISpot and ISR-immune reactive responses. The regimen was well-tolerated with minimal Grade 3–4 toxicities.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Combination immunotherapy regimens such as this could offer a promising alternative to other treatments with significant toxicities for select patients.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756968/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048793","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":"Red blood cell transfusion and the use of intravenous iron in iron-deficient patients presenting to the emergency department","authors":"Kevin Thomas,&nbsp;Arvand Barghi,&nbsp;Robert Balshaw,&nbsp;Emily Rimmer,&nbsp;Murdoch Leeies,&nbsp;Donald S. Houston,&nbsp;Allan Garland,&nbsp;Ryan Zarychanski,&nbsp;Brett L. Houston","doi":"10.1002/jha2.1061","DOIUrl":"10.1002/jha2.1061","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and aims</h3>\u0000 \u0000 <p>Red blood cell transfusions are often used to treat iron-deficient patients in the emergency department (ED), while treatment with intravenous (IV) iron is preferred, as it increases hemoglobin concentration rapidly and durably. We aim to evaluate the incidence of iron deficiency anemia, frequency of blood transfusion and iron supplementation, and factors associated with blood transfusion in the ED.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We conducted a retrospective cohort study of adult patients presenting to the St. Boniface Hospital (Winnipeg, Canada) ED from 2014 to 2018. Electronic data obtained from the Emergency Department Information System and Laboratory Information Services databases identified patients presenting with iron deficiency anemia, defined as microcytic (mean corpuscular volume &lt; 75 fL) anemia (hemoglobin &lt; 120 g/L) with either a transferrin saturation &lt;20% or ferritin &lt; 30 µmol/L. Ferritin &gt; 100 µmol excluded iron deficiency anemia. The use of blood transfusions or iron supplementation was determined for each patient. Factors associated with blood transfusion were determined using logistic regression analyses.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 39,222 patients, 17,945 (45%) were anemic. In anemic patients, iron parameters were ordered in 1848 (10.3%) and iron deficiency anemia was diagnosed in 910 (5.1%). Ninety-five patients (10.4%) received one red blood cell unit, and 197 patients (21.6%) received ≥2 units. Oral iron and IV iron were prescribed for 64 (7.0%) and 14 (1.5%) patients, respectively. Hemoglobin concentration was the main determinant for treatment with blood transfusion.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Iron deficiency is underinvestigated among anemic patients presenting to the ED. The only clinical factor associated with red blood cell transfusion in the ED was hemoglobin level, irrespective of symptoms or clinical stability.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756976/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143049018","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":"Increased serum B-cell maturation antigen levels evaluated with an Elecsys-based serum B-cell maturation antigen assay have a negative prognostic value in patients with newly diagnosed multiple myeloma","authors":"Evangelos Terpos,&nbsp;Ioannis Ntanasis-Stathopoulos,&nbsp;Panagiotis Malandrakis,&nbsp;Despina Fotiou,&nbsp;Magdalini Migkou,&nbsp;Evangelos Eleutherakis-Papaiakovou,&nbsp;Foteini Theodorakakou,&nbsp;Maria Roussou,&nbsp;Maria Gavriatopoulou,&nbsp;Meletios A Dimopoulos,&nbsp;Efstathios Kastritis","doi":"10.1002/jha2.889","DOIUrl":"10.1002/jha2.889","url":null,"abstract":"<p>Background: Serum B-cell maturation antigen (sBCMA) levels have emerged as a potential biomarker for disease monitoring in multiple myeloma (MM) with prognostic value.</p><p>Methods: Herein, we evaluated the sBCMA levels in 166 patients with newly diagnosed MM with an Elecsys-based sBCMA assay.</p><p>Results: Increased sBCMA levels at diagnosis were correlated with inferior survival outcomes in terms of both progression-free and overall survival. In a subset of patients with available samples at the time of disease progression, there was a trend for decreasing sBCMA values.</p><p>Conclusion: Sequential evaluation of sBCMA in prospective studies will determine the value of incorporating sBCMA measurement in clinical practice.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756978/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048883","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":"Real-world characteristics and outcomes of patients with multiple myeloma receiving second-line treatment in England","authors":"Sally Moore,&nbsp;Laura Cornic,&nbsp;Christina-Jane Crossman-Barnes,&nbsp;Sophie Jose,&nbsp;Zeyad Khalaf,&nbsp;Kwee Yong,&nbsp;Megan Soutar,&nbsp;Philip Woods","doi":"10.1002/jha2.1058","DOIUrl":"10.1002/jha2.1058","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Despite recent advances in first-line therapies for multiple myeloma (MM), most patients relapse or become refractory, underscoring the need for effective second-line (2L) regimens for relapsed/refractory MM (RRMM).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This study describes the real-world baseline characteristics, treatment patterns and clinical outcomes of adult patients diagnosed with MM between 2013 and 2020 using data collated by the National Cancer Registration and Analysis Service (NCRAS) of the National Health Service in England. The study cohorts were broadly aligned to the eligibility criteria of the ongoing DREAMM-7 (D7) and DREAMM-8 (D8) clinical trials. We focus on lenalidomide-exposed/refractory patients who received daratumumab–bortezomib–dexamethasone (DaraVd) at 2L in both cohorts.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In the D7-like cohort, the lenalidomide-exposed (<i>n </i>= 282) and lenalidomide-refractory (<i>n </i>= 143) patients who received DaraVd at 2L had a median (95% confidence interval [CI]) time to next treatment or death (TTNTD) of 15.1 (12.6–22.4) and 10.3 (7.4–13.9) months, respectively. In the D8-like cohort, the lenalidomide-exposed (<i>n </i>= 269) and lenalidomide-refractory (<i>n </i>= 148) patients who received DaraVd at 2L had a median (95% CI) TTNTD of 14.5 (11.7–19.7) and 10.0 (7.3–13.7) months, respectively.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Patients with RRMM in England receiving DaraVd at 2L have poor clinical outcomes, highlighting the urgent need for new therapies, particularly for lenalidomide-refractory patients.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756965/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143049014","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":"Terminal deoxynucleotidyl transferase-positive high-grade B-cell lymphoma with MYC and BCL2 rearrangements transformed from follicular lymphoma","authors":"Radu Chiriac,&nbsp;Lucile Baseggio,&nbsp;Marie Donzel","doi":"10.1002/jha2.1060","DOIUrl":"10.1002/jha2.1060","url":null,"abstract":"&lt;p&gt;A 48-year-old man with a 2-year history of classical follicular lymphoma (according to the 5th WHO classification) [&lt;span&gt;1&lt;/span&gt;] involving the axillary lymph nodes achieved complete remission after six cycles of obinutuzumab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. By the end of the third cycle of maintenance therapy with obinutuzumab, the patient presented with epigastric and left hypochondrial pain while in complete remission for 8 months.&lt;/p&gt;&lt;p&gt;Laboratory studies revealed a lactate dehydrogenase level of 2000 U/L (reference range: 135–235 U/L) and mild anemia (90 g/L). However, the peripheral blood smear showed 10% atypical intermediate-sized lymphomatous cells, characterized by nuclei with oval to irregular contours, finely stippled chromatin, variable nucleoli, and intensely basophilic cytoplasm with numerous vacuoles (Figure 1A). Staging bone marrow was negative. Peripheral blood flow cytometry showed a kappa-restricted population of mature B-cells that were CD45+, CD19+, CD10+, CD5-, and CD20- (Figure 1B). To evaluate the abdominal pain, &lt;sup&gt;18&lt;/sup&gt;F-fluorodeoxyglucose (&lt;sup&gt;18&lt;/sup&gt;F-FDG) positron emission tomography/computed tomography revealed a left retroperitoneal solid mass with increased &lt;sup&gt;18&lt;/sup&gt;F-FDG uptake, an SUVmax of 20, and measuring 6 × 16 cm (Figure 1C).&lt;/p&gt;&lt;p&gt;Mass biopsy revealed a predominance of monomorphic medium-sized blastoid cells with scant basophilic cytoplasm, round nuclei, and conspicuous nucleoli. Mitotic figures were easily observed (Figure 1D). The neoplastic cells were positive for B-cell markers, including PAX5 and CD19, but negative for CD20. They exhibited a germinal center phenotype (CD10+, BCL6-, and MUM1+) and overexpressed both c-MYC and BCL2. Diffuse terminal deoxynucleotidyl transferase (TdT) expression and monotypic surface immunoglobulin light chain expression were also observed. CD5, CD34, P53, and Epstein–Barr virus-encoded RNA in situ hybridization were negative. The Ki-67 proliferation index was 70%. Fluorescence in-situ hybridization performed with break-apart probes on tissue samples showed &lt;i&gt;MYC&lt;/i&gt; (80%) and &lt;i&gt;BCL2&lt;/i&gt; (90%) rearrangements, with no &lt;i&gt;BCL6&lt;/i&gt; rearrangement (Figure 1D, insets).&lt;/p&gt;&lt;p&gt;Ifosfamide and etoposide-based chemotherapy were initiated, followed by anti-CD19 chimeric antigen receptor T-cell infusion, which was initially well-tolerated; however, a recurrence developed 3 months later. He was switched to dexamethasone, high-dose cytarabine, and oxaliplatin but developed tumor lysis syndrome with renal failure despite prophylaxis. He experienced progressive disease involving the kidney, lower retroperitoneum, extraperitoneal space, and testis, ultimately dying 7 months after diagnosis.&lt;/p&gt;&lt;p&gt;This case describes a complex example of an aggressive TdT-positive high-grade B-cell lymphoma (HGBCL), with &lt;i&gt;MYC&lt;/i&gt; and &lt;i&gt;BCL2&lt;/i&gt; rearrangements transformed from follicular lymphoma marked by significant tissue involvement and a concurrent l","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"5 6","pages":"1351-1353"},"PeriodicalIF":0.0,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11647685/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142848238","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}