EJHaem最新文献

{"title":"Safety and Efficacy of Busulphan Based on Dosing Patterns in the Real-World Management of Myeloproliferative Neoplasms","authors":"Ali Mahdi,&nbsp;Alexandros Rampotas,&nbsp;Patrick Roberts,&nbsp;Joanna Stokes,&nbsp;Eamon Mahdi,&nbsp;Ruth Witherall,&nbsp;Deepak Mannari,&nbsp;Naheed Ibrahim,&nbsp;Georgina Naylor,&nbsp;Mamta Garg,&nbsp;Imran Manjra,&nbsp;Paula Glancy,&nbsp;George Katis,&nbsp;Sahil Bhagat,&nbsp;Jason Coppell,&nbsp;Andrew McGregor,&nbsp;Rebecca Frewin,&nbsp;Nauman M. Butt","doi":"10.1002/jha2.1097","DOIUrl":"https://doi.org/10.1002/jha2.1097","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Myeloproliferative neoplasms (MPNs), such as polycythaemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF), are primarily treated by managing blood counts to reduce the thrombotic risk using cytoreductive agents. Busulphan, an oral alkylating agent, has been historically used for MPN management due to its myelosuppressive effects, but concerns about its risk of leukaemic transformation have limited its use.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This real-world retrospective study evaluated the safety and efficacy of busulphan in 115 MPN patients across 13 UK hospitals. Responses in patients with ET and PV only were assessed using European LeukemiaNet (ELN) criteria.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>With a median age of 78 years, the overall response rate was 78.1%, with 29% of PV and 18% of ET patients achieving complete responses. Dosing regimens were similarly distributed between repeated single doses of busulphan (31%), courses of treatment lasting 1–4 weeks (30%) and continuous therapy for more than 4 weeks (35%). No cases of disease progression to acute leukaemia or myelofibrosis were recorded during the median follow-up of 23 months. Adverse events were infrequent, with fatigue and cytopaenia being the most common (4% each).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Busulphan demonstrated a favourable safety profile and is a viable cytoreductive option, particularly for elderly patients who are intolerant to hydroxycarbamide.</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":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1097","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143645724","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":"Bortezomib, Rituximab and Dexamethasone Regimen (BDR) in Waldenström Macroglobulinaemia: A Retrospective Real-World Analysis","authors":"Thomas Hueso,&nbsp;Grégory Lazarian,&nbsp;Paul Chauvet,&nbsp;Adrien Chauchet,&nbsp;Ramy Rahmé,&nbsp;Sabine Brechignac,&nbsp;Vincent Lévy,&nbsp;Salomon Manier,&nbsp;Damien Roos-Weil,&nbsp;David Ghez,&nbsp;Claude Gardin,&nbsp;Fanny Baran-Marszak,&nbsp;Eric Durot,&nbsp;Pierre Morel,&nbsp;Thorsten Braun","doi":"10.1002/jha2.70019","DOIUrl":"https://doi.org/10.1002/jha2.70019","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>We retrospectively analysed bortezomib–dexamethasone–rituximab (BDR) combination in patients with Waldenström macroglobulinaemia (WM) in a real world setting.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A total of 87 patients were included: 49 patients (56%) were treated in frontline, 22 (25%) in second line and 16 (19%) in third or further line settings. A log-rank test was used to compare overall and event-free survival (OS and EFS) whereas a Gray's test was performed to compare cumulative incidence of deaths and relapse (CID and CIR) according to the IPSS-WM groups, MYD88/CXCR4 mutational status and line of therapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The overall response rate was 88% with five patients (6%) achieving complete response, 20 (24%) very good partial response, 38 (45%) partial response and 11 (13%) minor response. The median time to achieve the best overall response was 9 months and the median EFS was 33 months for whole cohort. Patients treated in third line or further relapse settings had significantly lower median EFS compared to those treated in second- or first-line setting (13 vs. 36 vs. 47 months, respectively, <i>p</i> = 0.01) and a higher 7-year CID (50% vs. 13% vs. 12% respectively, <i>p</i> = 0.02). Among patients for whom mutational status was available, MYD88<i>^L265P</i> mutation or double mutation MYD88/CXCR4 did not influence OS or EFS. Severe peripheral neurotoxicity affected 7% of patients and 52 (62%) patients relapsed or died as result of WM whereas 21 patients (24%) died of unrelated causes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>BDR represents an interesting chemo-free, fixed duration regimen for patients in first or second line, regardless of mutational status.</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":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70019","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143645723","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":"Underrecognised Functional Hyposplenism Associated With Chronic Graft-Versus-Host Disease: A Case Report","authors":"Kaori Uchino,&nbsp;Yuya Nakagami,&nbsp;Megumi Enotomoto,&nbsp;Nozomi Shimizu,&nbsp;Kenichi Kondo,&nbsp;Takahiro Yamamoto,&nbsp;Yukie Sugita,&nbsp;Hideshige Seki,&nbsp;Sakura Saigusa,&nbsp;Yusuke Iida,&nbsp;Saki Shinohara,&nbsp;Soichi Takasugi,&nbsp;Tomohiro Horio,&nbsp;Satsuki Murakami,&nbsp;Shohei Mizuno,&nbsp;Kazuhiro Ikegame,&nbsp;Ichiro Hanamura,&nbsp;Akiyoshi Takami","doi":"10.1002/jha2.70017","DOIUrl":"https://doi.org/10.1002/jha2.70017","url":null,"abstract":"&lt;p&gt;Haematopoietic stem cell transplantation (HSCT) is a curative treatment for haematologic malignancies; however, complications such as graft-versus-host disease (GVHD) and infections remain major causes of morbidity and mortality [&lt;span&gt;1-4&lt;/span&gt;]. Functional hyposplenism, often linked to chronic GVHD, is an underrecognised complication of HSCT that increases the risk of severe infections caused by encapsulated organisms [&lt;span&gt;5&lt;/span&gt;]. This report describes a case of a patient with chronic GVHD who developed progressive splenic atrophy and persistent Howell–Jolly bodies (HJBs) in peripheral blood smears, indicative of functional hyposplenism [&lt;span&gt;6-9&lt;/span&gt;]. The case highlights the importance of blood smear examinations and imaging studies for early diagnosis and management of this overlooked complication.&lt;/p&gt;&lt;p&gt;A 44-year-old man with acute myeloid leukaemia (AML) harbouring &lt;i&gt;NPM1&lt;/i&gt; and &lt;i&gt;DNMT3&lt;/i&gt; mutations underwent allogeneic HSCT from an HLA-identical sibling donor after induction therapy, which caused persistent pancytopenia despite achieving complete remission. Myeloablative conditioning with cyclophosphamide and busulphan was administered, and GVHD prophylaxis included cyclosporine (CSA) and short-term methotrexate. Engraftment was achieved with full donor chimerism, and no acute GVHD or initial complications occurred, allowing CSA tapering.&lt;/p&gt;&lt;p&gt;On day 185 post-HSCT, cryptogenic organising pneumonia (COP) developed, requiring prednisolone (PSL; 0.5 mg/kg/day). PSL tapering followed symptom improvement. However, oral lichenoid lesions and elevated liver enzyme levels on day 380 led to a diagnosis of chronic GVHD. PSL was increased to 10 mg/day, but tapering PSL and CSA proved challenging due to worsening GVHD symptoms.&lt;/p&gt;&lt;p&gt;At 2.5 years post-HSCT, HJBs were detected in peripheral blood smears (Figure 1), and computed tomography (CT) scans revealed significant splenic atrophy. The spleen volume, normal at HSCT (136 mL, Figure 2A), progressively declined—34 mL at 2.5 years (Figure 2B), 22 mL at 3.5 years (Figure 2C), 12 mL at 4.5 years (Figure 2D), and 8 mL at 5.5 years (Figure 2E).&lt;/p&gt;&lt;p&gt;This case highlights the need to recognise functional hyposplenism as a complication of chronic GVHD post-HSCT. The patient's progressive splenic atrophy, identified via routine CT imaging, and persistent HJBs in peripheral blood smears indicated functional hyposplenism. Despite its clinical significance, functional hyposplenism is often underdiagnosed due to overlooked splenic atrophy in radiology reports and the limited use of blood smear examinations.&lt;/p&gt;&lt;p&gt;The spleen plays a key role in immune defence and erythrocyte filtration, and its dysfunction increases susceptibility to infections by encapsulated microorganisms [&lt;span&gt;7, 10&lt;/span&gt;]. This patient received PCV13 and PPV23 vaccinations and prophylactic antibiotics, including trimethoprim-sulphamethoxazole and levofloxacin, which effectively prevented pneumococcal infections. Such prev","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70017","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143612395","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":"Characterization of Immunoglobulin Heavy Locus Rearrangements in Molecular Subtypes of Childhood B-Cell Precursor Acute Lymphoblastic Leukemia","authors":"Guilherme Navarro Nilo Giusti,&nbsp;Patrícia Yoshioka Jotta,&nbsp;Caroline de Oliveira Lopes,&nbsp;Natacha Azussa Migita,&nbsp;Amilcar Cardoso de Azevedo,&nbsp;Sílvia Regina Brandalise,&nbsp;João Meidanis,&nbsp;José Andrés Yunes","doi":"10.1002/jha2.70003","DOIUrl":"https://doi.org/10.1002/jha2.70003","url":null,"abstract":"<p>Biased <i>IGH</i> VDJ recombination has been previously described in childhood B-cell precursor acute lymphoblastic leukemia (BCP-ALL), although its causes are not yet fully understood. This study assesses differential features in 565 <i>IGH</i> clonotypes from BCP-ALL molecular subsets against 560 clonotypes from bone marrow donors. Leukemia clonotypes were enriched for <i>IGHV6-1</i> segments in the KMT2A rearranged and B-other subtypes, while <i>IGHV3-23</i> was enriched in TCF3::PBX1. ETV6::RUNX1 presented a topological gap in the usage of central IGHV segments. BCP-ALL also presented shorter CDR3 regions, higher GC content, and lower productivity. Interestingly, productive clonotypes tended to be absent after induction therapy.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143595044","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":"Invasive Fungal Disease Associated With Targeted Agents for Acute Myeloid Leukaemia: A Systematic Review","authors":"Samir Agrawal,&nbsp;Anjaneya Bapat,&nbsp;Christopher P. Eades,&nbsp;Shreyans Gandhi","doi":"10.1002/jha2.1105","DOIUrl":"https://doi.org/10.1002/jha2.1105","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To examine the incidence of invasive fungal disease (IFD) in patients receiving targeted agents for acute myeloid leukaemia (AML).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Literature for this systematic review was identified through a PubMed search in April 2024, using AML, IFD and targeted therapy terms. The following filters were applied: published in the last 10 years and published in English.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The PubMed search yielded 54 results, of which 16 were deemed relevant for inclusion. Four additional references were identified through manual searches. The majority of publications focused on the incidence of IFD during treatment with targeted agents; the remainder focused on the efficacy of targeted treatments and reported IFD as an adverse event. Most publications were retrospective analyses. Prophylaxis use and agents differed across studies. In several studies, IFD incidence was above the 8% threshold identified for anti-mould prophylaxis. <i>Aspergillus</i> was the most commonly reported pathogen, and most IFD cases occurred in the lungs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>IFD is relatively common among patients with AML receiving targeted therapies, despite the use of prophylaxis. Prospective studies with detailed IFD reporting, together with large epidemiological studies, are required to better understand the risk factors for, and incidence and nature of IFD in this patient population.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1105","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143595043","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":"Lisocabtagene Maraleucel for Richter's Transformation—A Case Series","authors":"Courtney J. Smith,&nbsp;Anmol Goyal,&nbsp;Brian R. Smith,&nbsp;Dasom Lee,&nbsp;Alexandria Jensen,&nbsp;Jonathan Alexander,&nbsp;Melody Smith,&nbsp;Matthew Frank,&nbsp;Saurabh Dahiya,&nbsp;David Miklos,&nbsp;Sushma Bharadwaj","doi":"10.1002/jha2.70011","DOIUrl":"https://doi.org/10.1002/jha2.70011","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Richter's transformation (RT) from chronic lymphocytic leukemia (CLL) to lymphoma carries poor prognosis. This case series examines the efficacy of lisocabtagene maraleucel (liso-cel) in six RT patients, highlighting the impact of concurrent ibrutinib therapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Six patients were with RT who received liso-cel from were included in this single institution case series. Clinical data related to efficacy, safety, CAR-T expansion kinetics, and measurable residual disease were collected.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The best overall response was 83.3%. The four patients who received ibrutinib concurrent with liso-cel therapy continue to show MRD-negative complete response till date. None experienced severe (grade 3+) cytokine release syndrome while 1 had severe (grade 3+) immune-effector cell-associated neurotoxicity syndrome (ICANS). All patients were noted to have in vivo CAR expansion.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This series of real cases suggests liso-cel with concurrent ibrutinib may be a promising treatment for RT, warranting further exploration.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70011","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143581676","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":"Central nervous system relapse of primary cutaneous anaplastic large cell lymphoma: A case report","authors":"Satoshi Mitsuyuki,&nbsp;Sayaka Okazaki,&nbsp;Satoru Mukai,&nbsp;Ai Matsuura,&nbsp;Yumiko Yasuhara,&nbsp;Aya Tanaka,&nbsp;Koichi Oshima,&nbsp;Kazuo Hatanaka","doi":"10.1002/jha2.1082","DOIUrl":"https://doi.org/10.1002/jha2.1082","url":null,"abstract":"<p>Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) has a high relapse rate. However, it typically remains confined to the skin and has a favorable long-term prognosis. We describe a case of PC-ALCL that experienced a relapse in the central nervous system (CNS). The patient presented with somatosensory abnormalities in the extremities after local treatment of skin lesions and was diagnosed with CNS relapse of PC-ALCL. Methotrexate, procarbazine, and vincristine therapy, and alternating brentuximab vedotin, followed by autologous hematopoietic stem cell transplantation (ASCT) cured the CNS lesions, whereas the skin lesions relapsed early. PC-ALCL could relapse in the CNS; systemic chemotherapy and ASCT may be effective.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1082","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143554844","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":"Extramedullary Relapse of Acute Promyelocytic Leukaemia (APL) at an Unusual Site (External Auditory Canal)","authors":"Prathima Iyer,&nbsp;Carole Hudson,&nbsp;Kanwar Sohail,&nbsp;Loredanna Mihailescu,&nbsp;Simon Bolam","doi":"10.1002/jha2.70013","DOIUrl":"https://doi.org/10.1002/jha2.70013","url":null,"abstract":"<p>A 55-year-old lady diagnosed with APL-M3 variant in 2021 and treated with AIDA regimen to complete remission (CR1) and negative MRD. She maintained MRD negative results at 3 months post treatment.</p><p>Six months after treatment, she presented with persistent feeling of blocked right ear canal and otalgia. She had an inflamed right ear canal and difficulties to visualise the right tympanic membrane due to a polypoidal lesion. She did not respond to initial antibiotic treatment and had progressed to decreased auditory acuity with persistent ear pain and development of right cervical and periauricular lymphadenopathy.</p><p>Her blood counts were within normal range at this time. MRI head demonstrated in FLAIR coronal (Figure 1A) and T2 axial (Figure 1B) images, a soft tissue in right external auditory canal. The biopsy of this mass showed monomorphic cells with high nucleocytoplasmic ratio. On immunohistochemistry, these were positive for CD117(C), MPO (D), CD33 and CD68. A contemporaneous bone marrow biopsy was in morphological remission, however the flowcytometry found a population of CD33, CD117 and MPO positive cells and positive MRD of 18.5%. Cytogenetics (E) showed ongoing presence of the t (15, 17). She had a lumbar puncture and flowcytometry results of CSF analysis ruled out CNS involvement.</p><p>She commenced arsenic and ATRA treatment and achieved PET and bone marrow remission post induction. She continued with 3 further consolidation cycles, followed by consolidation with an autologous stem cell transplant and at D+100 continues to be MRD negative in bone marrow and radiological remission on PET.</p><p>This is an unusual extramedullary relapse of APL (external auditory canal), while the blood counts were normal and bone marrow was in morphological remission. This reiterates the importance of MRD monitoring and low threshold to investigate unusual or persistent auditory symptoms, as these may be the first presentation of relapse of the disease. Extramedullary relapse is infrequent in APL and relapse in auditory canal has been reported as an unusual site of relapse [<span>1, 2</span>], considering that usually the extramedullary relapses happen in skin or CNS.</p><p>The authors declare no conflicts of interest.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70013","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143554845","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":"Haploidentical Allogeneic Stem Cell Transplantation as a Superior Alternative for Patients With Mismatch Donors—A Single Center Experience in 152 Patients","authors":"Paul Jäger,&nbsp;Benno Biermann,&nbsp;Nora Liebers,&nbsp;Felicitas Schulz,&nbsp;Ben-Niklas Baermann,&nbsp;Sören Twarock,&nbsp;Stefanie Geyh,&nbsp;Kathrin Nachtkamp,&nbsp;Patrick Tressin,&nbsp;Annika Kasprzak,&nbsp;Felix Matkey,&nbsp;Titus Watrin,&nbsp;Malika El Yaouti,&nbsp;Ulrich Germing,&nbsp;Sascha Dietrich,&nbsp;Guido Kobbe","doi":"10.1002/jha2.70012","DOIUrl":"https://doi.org/10.1002/jha2.70012","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Allogeneic hematopoietic stem cell transplantation (allo-SCT) is a key treatment for hematologic malignancies, but donor selection impacts outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In a cohort of 152 patients undergoing allo-SCT from 2012 to 2023, haploidentical donors with post-transplant cyclophosphamide (PTCy) showed superior survival compared to 9/10 mismatched unrelated donors (MMUD). Cox regression analysis revealed that patients not in complete remission (CR) before transplantation particularly benefited from haplo donors, while those with 9/10 MMUD and lacking CR had worse outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>These results highlight the importance of donor selection, suggesting that haplo donors with PTCy may be preferable for patients not in CR, necessitating alternative approaches for others.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Clinical 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":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.70012","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143533509","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":"Overall and Cause-Specific Mortality Among Patients With Cutaneous T-Cell Lymphoma in the United States","authors":"Lauren Shea,&nbsp;Mayur Narkhede,&nbsp;Karthik Chamarti,&nbsp;Tina Gao,&nbsp;Amitkumar Mehta,&nbsp;Gaurav Goyal","doi":"10.1002/jha2.1099","DOIUrl":"https://doi.org/10.1002/jha2.1099","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Little is known about long-term outcomes and causes of death for individuals with cutaneous T-cell lymphoma.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We used SEER-18 registry data to examine outcomes among 9886 adults with mycosis fungoides (MF), Sézary syndrome (SS), primary cutaneous anaplastic large cell lymphoma (pcALCL), and subcutaneous panniculitis-like T-cell lymphoma (SPTCL) diagnosed from 2000 to 2018. We calculated overall survival (Kaplan–Meier method), standardized mortality ratios (SMRs), absolute excess risk (AER) of death, and cumulative incidence of cause-specific mortality.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Individuals with CTCL were at increased risk of all-cause mortality relative to age-matched controls, with SMR ranging from 1.57 (95% CI: 1.49–1.65) in MF to 5.61 (95% CI: 4.65–6.7) in SS. This was true even for those who initially presented with early-stage disease. After a median follow-up of 64 months, the cumulative incidence of lymphoma-related death was 16.5%, compared to 10.5% other causes, 9.6% cardiovascular, 9.1% second primary malignancy, 1.8% infection, and 1.1% unknown cause. People diagnosed with CTCL were at higher risk of mortality due to leukemia and infectious causes than control populations, but secondary causes made overall minor contributions to total mortality.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This population-level analysis revealed that individuals with CTCL were at increased risk of all-cause mortality relative to age-matched controls and that lymphoma remained a significant cause of death even in those presenting with early-stage disease.</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":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jha2.1099","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143533508","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}