EJHaem最新文献

{"title":"Central nervous system posttransplant lymphoproliferative disorder following allogeneic hematopoietic stem cell transplantation successfully treated with combination therapy of acalabrutinib and immunochemotherapy: A case report and literature review","authors":"Peihao Zheng,&nbsp;Teng Xu,&nbsp;Xiaona Zuo,&nbsp;Xiaoyan Ke,&nbsp;Kai Hu","doi":"10.1002/jha2.1078","DOIUrl":"10.1002/jha2.1078","url":null,"abstract":"<p>Here, we report a case of Epstein-Barr virus-positive central nervous system-post-transplant lymphoproliferative disorder (CNS-PTLD) patient who failed to achieve complete metabolic remission (CMR) after successively trying a methotrexate-based regimen combined with orelabrutinib or whole-brain radiotherapy and encountered intracranial hemorrhage during orelabrutinib treatment. Ultimately, the patient achieved CMR after one cycle of acalabrutinib in combination with temozolomide, teniposide, liposomal doxorubicin, dexamethasone, and rituximab (TEDDi-R). Following another cycle of TEDDi-R treatment, he has been receiving acalabrutinib maintenance up to now and remained in CMR. The case may provide an effective treatment option for CNS-PTLD patients in clinical practice.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756970/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048792","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 long-term risk of immune-related conditions in survivors of diffuse large B-cell lymphoma: A Danish nationwide registry study","authors":"Laura Schou Pedersen,&nbsp;Nadja Nørholm Klausen,&nbsp;Jonas Faartoft Jensen,&nbsp;Emilis Danielsen Bacevičius,&nbsp;Peter Brown,&nbsp;Judit Mészáros Jørgensen,&nbsp;Thomas Stauffer Larsen,&nbsp;Christian Bjørn Poulsen,&nbsp;Michael Roost Clausen,&nbsp;Robert Schou Pedersen,&nbsp;Anne Ortved Gang,&nbsp;Rasmus Westermann,&nbsp;Salome Kristensen,&nbsp;Lene Wohlfahrt Dreyer,&nbsp;Tarec Christoffer El-Galaly,&nbsp;Lasse Hjort Jakobsen","doi":"10.1002/jha2.1070","DOIUrl":"10.1002/jha2.1070","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>There is limited knowledge of the long-term effects on the immune system after treatment for diffuse large B-cell lymphoma (DLBCL).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This study included DLBCL patients from the Danish Lymphoma Registry who obtained complete remission (CR) after (R)-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone)-like immunochemotherapy. Each R⁺ CHOP-like treated patient was matched to five comparators from the Danish background population and furthermore compared to R⁻ CHOP-like treated patients. Incidence rate ratios (IRRs) and risk differences (RDs) were calculated for a wide range of infections, autoimmune conditions, and immune deficiencies (AC-IDs) combined and by subtypes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>R⁺ CHOP-like treated patients had a higher risk of infections overall (IRR 1.5, 95% confidence interval [CI] 1.4–1.7: 10-year RD 5.0%, 95% CI 2.2%–7.8%) and for a majority of the subtypes than matched comparators. Likewise, they had a higher risk of AC-IDs overall (IRR 1.4, 95% CI 1.1–1.7; RD 0.8%, 95% CI 0.7%–2.2%) than matched comparators, however only of clinical relevance for three subtypes; autoimmune diseases of the endocrine system, sarcoidosis and immune deficiencies. The addition of rituximab to CHOP-like therapy did not alter the incidence rates (IR) of infections overall (IRR 1.1, 95% CI 0.9–1.3) or AC-IDs overall (IRR 0.8, 95% CI 0.5–1.3) compared to CHOP-like therapy alone, although the IR for respiratory infections was significantly elevated (IRR 1.5, 95% CI 1.1–2.1). However, an increased use of IVIG treatment was observed among R⁺ CHOP survivors.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>R-CHOP-like treated patients face an increased risk of infections and AC-IDs overall compared with the background population. The risk of infections and AC-IDs did not change overall after the addition of rituximab to CHOP, however, an increased risk of respiratory infections is notable. These findings could highlight the need for expanded vigilance and prophylaxis strategies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756995/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048972","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":"Single-center off-label benralizumab use for refractory hypereosinophilic syndrome demonstrates satisfactory safety and efficacy","authors":"Yvonne Veltman,&nbsp;Anna M. Aalbers,&nbsp;Maud A. W. Hermans,&nbsp;Pim G. N. J. Mutsaers","doi":"10.1002/jha2.1014","DOIUrl":"10.1002/jha2.1014","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Benralizumab is an interleukin 5-receptor-blocking drug registered for the treatment of eosinophilic asthma. It has proven efficient and safe in a small phase-II trial in hypereosinophilic syndrome and is currently being investigated in a larger, randomized phase-III trial. We report on real-world experience with benralizumab in 15 patients with severe Hypereosinophilic syndrome (HES) that were refractory to other treatments or on unacceptable steroid doses.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Fifteen patients with severe HES were treated with compassionate need benralizumab. The measured endpoints were a reduction in peripheral eosinophil count, a reduction of corticosteroid use, adverse events, and clinical response.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>All subgroups of HES were represented in this cohort and in the case of reactive HES, treatment of the primary cause did not lead to resolution of the eosinophilia. The median time of follow-up was 38 months. Twelve patients reached a normalized peripheral eosinophil count (&lt; 0.05 × 109/L), while the remaining three patients also had a significant reduction from baseline.</p>\u0000 \u0000 <p>Of the eight patients initially treated with steroids, five patients were off steroids completely, and three patients had reduced dosages. Eight patients experienced complete symptom resolution, and five partial resolution. No serious adverse events were observed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In conclusion, benralizumab is safe and effective for the treatment of HES.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756971/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143049028","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":"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}