European Journal of Haematology最新文献

{"title":"Improved Outcomes of Myeloma Cast Nephropathy in Newly Diagnosed Multiple Myeloma With Modern Anti-Myeloma Therapies.","authors":"Michael Sang Hughes, Metodi Balev, Jai Radhakrishnan, Divaya Bhutani, Markus Mapara, Suzanne Lentzsch, Rajshekhar Chakraborty","doi":"10.1111/ejh.14403","DOIUrl":"https://doi.org/10.1111/ejh.14403","url":null,"abstract":"<p><p>Myeloma cast nephropathy (MCN) is a driver of renal failure in newly diagnosed multiple myeloma (NDMM) and has been historically associated with increased early mortality. Since patients with moderate to severe renal insufficiency are typically excluded from trials, we performed a retrospective study to characterize modern-era outcomes in MCN. We reviewed 274 consecutive NDMM patients from 2017 to 2023 at an academic center and identified 46 patients (16.8%) with MCN. Among them, 96% had received bortezomib and 67% anti-CD38⁺ monoclonal antibody in frontline therapy. As per the International Myeloma Working Group criteria, the renal overall response rate was 76.1% (35/46), and the renal complete response (CR) rate was 32.6% (15/46) at 6 months. Overall survival (OS) at 6 months did not differ between MCN (100%) and controls (98.2%). At a median follow-up of ~3 years, the mean MCN OS was within 7 months of control (p = 0.039) by equivalence testing. Most involved free light chain (iFLC) and proteinuria reduction occurred within 1 month of treatment (83.1%, 3.9 g/d, respectively). In summary, we report excellent 6-month renal recovery without early mortality in MCN patients with modern anti-myeloma therapies. Prospective studies focused on MCN are urgently needed to further improve the renal CR rate.</p>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482489","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Case-Driven Multi-Omics Analysis for Longitudinal Ibrutinib Response Evaluation of Patients With Chronic Lymphocytic Leukemia.","authors":"Sólja Remisdóttir Veyhe, Marcus Høy Hansen, Oriane Cédile, Michael Boe Møller, Mie Kiszka Nielsen, Mads Thomassen, Karen Juul-Jensen, Henrik Frederiksen, Karen Dybkær, Charlotte Guldborg Nyvold","doi":"10.1111/ejh.14397","DOIUrl":"https://doi.org/10.1111/ejh.14397","url":null,"abstract":"<p><p>Patients with chronic lymphocytic leukemia (CLL) undergoing ibrutinib treatment often experience incomplete response, yet the molecular level underlying clonal inertia remains to be explored. We investigated the molecular and clinical dynamics of CLL during 16 months of ibrutinib monotherapy by analyzing blood samples from two patients who continued having CLL cells in the peripheral blood during treatment. At diagnosis, the clonal burden within the B cell compartment was found to be 55% (pt1) and 86% (pt2) for the dominant clones. At 16 months following treatment these clones still constituted 66% and 89%, respectively. Utilizing multi-omic methodologies at the DNA and RNA levels, including single-cell transcriptomics, we aimed to establish a comprehensive framework for multi-omics analysis for longitudinal ibrutinib response evaluation. The presented study revealed genomically stable disease during ibrutinib treatment, but with intensified expression of genes involved in pathways related to apoptosis, cellular stress response, and canonical NF-κB signaling from diagnosis to 16 months of treatment.</p>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Measurable Residual Disease in UBTF-TD Adult Acute Myeloid Leukemia.","authors":"Helena Castellet, Guillermo Ramil, Maite Carricondo, Guadalupe Oñate, Ana Garrido, Alícia Artigas, Marta Pratcorona, Olga Salamero, Albert Cortés-Bullich, Susana Vives, Mar Tormo, Martí Mascaró, David Gallardo, Josep Maria Marti Tutusaus, Antonio Garcia, Jorge Sierra, Jordi Esteve, Josep Nomdedéu","doi":"10.1111/ejh.14402","DOIUrl":"https://doi.org/10.1111/ejh.14402","url":null,"abstract":"","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482491","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes of Melflufen Treatment in Patients With Relapsed/Refractory Multiple Myeloma.","authors":"Shahrier Hossain, Clifton Mo, Sarah Patches, Houry Leblebjian, Kathryn Goodrich, Eileen Regan, Kathleen O'Neill, Kim Noonan, Paul G Richardson, Jacob Laubach","doi":"10.1111/ejh.14398","DOIUrl":"https://doi.org/10.1111/ejh.14398","url":null,"abstract":"<p><strong>Objective: </strong>Melphalan flufenamide (melflufen) plus dexamethasone is fully approved in Europe for patients with relapsed/refractory multiple myeloma (RRMM) with ≥ 3 prior lines of therapy. We analyzed the efficacy and safety of melflufen in the real-world setting.</p><p><strong>Methods: </strong>In this retrospective analysis, we examined baseline features, efficacy, and safety outcomes with melflufen plus dexamethasone in a cohort of 12 patients with heavily pre-treated RRMM at the Dana-Farber Cancer Institute, USA.</p><p><strong>Results: </strong>Patients had received a median of 5.5 prior lines of therapy. Three patients (25%) had extramedullary disease, three (25%) cytogenetically high-risk features, and five (42%) had received prior autologous stem cell transplantation. The overall response rate was 55% (complete response: three [27%], very good partial response: one [9%], partial response: two [18%] patients). Five patients (42%) had stable disease; one was non-evaluable. Adverse events (AEs) were mostly hematologic and proved manageable; two patients had Grade 2 infections. Reasons for melflufen discontinuation were progressive disease (42%), drug withdrawal from the United States market (33%), AEs (17%), and sudden death (8%) unrelated to treatment.</p><p><strong>Conclusions: </strong>Consistent with clinical trial data, melflufen had an expected safety profile with manageable toxicity and clinically meaningful efficacy in patients with RRMM treated in the real-world setting.</p>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Relapsed/Refractory Follicular Lymphoma: Current Advances and Emerging Perspectives","authors":"Giulio Caridà,&nbsp;Enrica Antonia Martino,&nbsp;Antonella Bruzzese,&nbsp;Daniele Caracciolo,&nbsp;Caterina Labanca,&nbsp;Francesco Mendicino,&nbsp;Eugenio Lucia,&nbsp;Virginia Olivito,&nbsp;Teresa Rossi,&nbsp;Antonino Neri,&nbsp;Ernesto Vigna,&nbsp;Pierfrancesco Tassone,&nbsp;Pierosandro Tagliaferri,&nbsp;Fortunato Morabito,&nbsp;Massimo Gentile","doi":"10.1111/ejh.14401","DOIUrl":"10.1111/ejh.14401","url":null,"abstract":"<p>Follicular lymphoma (FL) is a prevalent indolent non-Hodgkin lymphoma (NHL) characterized by a relapsing course and eventual refractoriness to therapy. Despite advancements in treatment, FL remains incurable, necessitating ongoing research into novel therapeutic strategies. This review provides a comprehensive overview of current standard treatments for relapsed or refractory (R/R) FL, including chemoimmunotherapy and stem cell transplantation, and delves into emerging therapies such as chimeric antigen receptor (CAR) T-cell therapy and bispecific antibodies. We discuss the efficacy and safety profiles of these innovative treatments, their integration into existing treatment paradigms, and the potential they hold in altering the natural history of FL. Additionally, we explore the challenges associated with these therapies, including accessibility, cost, and long-term management of adverse effects. By examining the evolving therapeutic landscape, this review aims to provide insights into future directions for achieving sustained remission and improving the quality of life in patients with R/R FL.</p>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":"114 5","pages":"775-784"},"PeriodicalIF":2.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ejh.14401","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Management of Persistent Hypereosinophilia","authors":"Grzegorz Helbig,&nbsp;Kacper Czachor","doi":"10.1111/ejh.14396","DOIUrl":"10.1111/ejh.14396","url":null,"abstract":"<p>Blood eosinophilia remains a common finding in the general population, whereas hypereosinophilia (HE) is extremely rare. Different non-hematologic and hematologic disorders may be accompanied by blood eosinophilia. Clinical manifestations of eosinophilia-related disorders range from mild to life-threatening. Given the various symptoms, a comprehensive approach and close multidisciplinary cooperation are strongly recommended. Hypereosinophilic syndromes (HES) encompass a complex group of disorders defined as persistent peripheral blood HE ≥ 1500/mm³ and end-organ damage. An initial step in a diagnostic algorithm of HE includes the evaluation of secondary, potentially easy-to-treat causes. This HES variant is called reactive and often remains beyond the interest of hematologists. The further evaluation for primary causes of HE relies on a combination of different specialized tests, which are mostly available in hematologic centers and include most of all morphologic assessments of peripheral blood and bone marrow, cytogenetics, and molecular studies. Using these sophisticated methods, one can diagnose the specific HES subtypes based on genetic findings and categorize them according to currently applicable classifications. The choice of therapy differs between HES variants, ranging from corticosteroids and anti-interleukin-5 monoclonal antibodies for not-molecularly defined variants to oral molecules directed against molecular targets. This review presents eosinophilia-related disorders from a hematologic perspective.</p>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":"114 5","pages":"763-774"},"PeriodicalIF":2.3,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ejh.14396","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Treatment-Free Remission Outcomes in a BCR::ABL1 Digital PCR Selected Clinical Cohort of CML Patients","authors":"C. Kockerols,&nbsp;P. J. M. Valk,&nbsp;P. Hogenbirk,&nbsp;I. Geelen,&nbsp;N. M. A. Blijlevens,&nbsp;J. J. W. M. Janssen,&nbsp;M. Hoogendoorn,&nbsp;S. Kersting,&nbsp;S. K. Klein,&nbsp;L. G. M. Daenen,&nbsp;M. Donker,&nbsp;P. A. W. te Boekhorst,&nbsp;K.-S. G. Jie,&nbsp;M. Corsten,&nbsp;M. J. Cruijsen,&nbsp;H. Levenga,&nbsp;W. M. Smit,&nbsp;M.-D. Levin,&nbsp;E. de Jongh,&nbsp;S. de Jonge,&nbsp;A. J. Vlot,&nbsp;M. F. Durian,&nbsp;J. J. Zwaginga,&nbsp;M. Mohlmann,&nbsp;T. J. Wustman,&nbsp;R. Blommers,&nbsp;J. J. Cornelissen,&nbsp;P. E. Westerweel,&nbsp;Collaborating Authors","doi":"10.1111/ejh.14368","DOIUrl":"10.1111/ejh.14368","url":null,"abstract":"<p>Approximately 40%–60% of patients reaching a stable deep molecular response during TKI treatment will maintain a state of remission after TKI discontinuation, denoted as treatment-free remission (TFR). Depth of molecular response assessed by <i>BCR::ABL1</i> digital PCR prior to TKI discontinuation has demonstrated its significance as a reliable predictive parameter for TFR. A clinically applicable prediction cutoff of 0.0023%IS has been established and externally validated. In this study, <i>BCR::ABL1</i> digital PCR, as most sensitive and stable assay of its kind, was investigated as a TFR prediction tool in the Netherlands, and evaluated for its predictive value to stop TKI treatment below the aforementioned cutoff. The primary endpoint of molecular recurrence (MolR, <i>BCR::ABL1</i> &gt; 0.1%IS) at 12 months was prospectively assessed. Overall, 67 discontinued patients below the set <i>BCR::ABL1</i> digital PCR cutoff were included. The overall MolR probability was 50% (95% CI, 36%–62%). In 38 patients treated for more than 6 years as commonly recommended as desirable treatment duration before TFR attempt, the MolR probability dropped to 36% (95% CI, 18%–50%). Patients attempting an early TKI discontinuation (treated for less than 6 years) had a high MolR probability of 76% (95% CI, 65%–89%). <i>BCR::ABL1</i> digital PCR was successfully used in Dutch clinical practice. Our study indicates that in patients with a low <i>BCR::ABL1</i> digital PCR result, a total TKI treatment duration of six or more years remains associated with a lower MolR rate and should generally be pursued. In patients treated for more than 6 years, <i>BCR::ABL1</i> digital PCR was capable to identify stop candidates with a higher probability of TFR success.</p>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":"114 5","pages":"900-907"},"PeriodicalIF":2.3,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ejh.14368","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143432806","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Completion of Distress Screening and Correlates of Distress Score in Patients With Multiple Myeloma at Initial Evaluation at a Transplant Center","authors":"Noel Estrada-Merly,&nbsp;James F. Wu,&nbsp;Liliana E. Pezzin,&nbsp;Mochamad M. Nataliansyah,&nbsp;Anita D'Souza","doi":"10.1111/ejh.14394","DOIUrl":"10.1111/ejh.14394","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>We describe the completion of the National Comprehensive Care Network's distress thermometer (DT) survey for multiple myeloma (MM), quantify, and identify factors influencing distress score.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We assessed DT completion for MM patients' first consultation at an academic center between January 1, 2015, and December 31, 2022. Multivariate logistic regression was conducted to identify factors associated with survey completion and distress.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 1011 new patients, 683 (68%) completed the DT survey. Noncompletion at initial consultation was associated with non-Hispanic Black patients [AOR 0.50 (95% CI 0.32–0.79), <i>p</i> = 0.003], socially vulnerable neighborhood residence [AOR 0.54 (95% CI 0.39–0.76), <i>p</i> = 0.0004], Karnofsky Performance Status &lt; 90 [AOR 0.60 (95% CI 0.43–0.86), <i>p</i> = 0.005], and recent years of consult [AOR 0.38, (95% CI 0.28–0.52), <i>p</i> &lt; 0.0001]. Nonrespondents were less likely to receive a subsequent autologous stem cell transplantation (71% vs. 79%, <i>p</i> &lt; 0.01). The median distress score among respondents was 3% with 22% reporting 0 distress. Distress was associated with female sex [AOR 1.48, (95% CI 1.07–2.04), <i>p</i> = 0.017] and stage [stage 2 vs. stage 1, AOR 1.76 (95% CI 1.20–2.57), <i>p</i> = 0.004].</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Disparities in nonrespondents of the DT survey at initial consultation suggest the limitations of relying on the screening tool to assess unmet needs among high-risk patients.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":"114 5","pages":"890-899"},"PeriodicalIF":2.3,"publicationDate":"2025-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143432803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sustained Improvement in Health-Related Quality of Life in Transplant-Ineligible Newly Diagnosed Multiple Myeloma Treated With Daratumumab, Lenalidomide, and Dexamethasone: MAIA Final Analysis of Patient-Reported Outcomes","authors":"Aurore Perrot,&nbsp;Thierry Facon,&nbsp;Torben Plesner,&nbsp;Saad Z. Usmani,&nbsp;Shaji Kumar,&nbsp;Nizar J. Bahlis,&nbsp;Cyrille Hulin,&nbsp;Robert Z. Orlowski,&nbsp;Hareth Nahi,&nbsp;Peter Mollee,&nbsp;Karthik Ramasamy,&nbsp;Murielle Roussel,&nbsp;Arnaud Jaccard,&nbsp;Michel Delforge,&nbsp;Lionel Karlin,&nbsp;Bertrand Arnulf,&nbsp;Ajai Chari,&nbsp;George Wang,&nbsp;Niodita Gupta-Werner,&nbsp;Shuchita Kaila,&nbsp;Huiling Pei,&nbsp;Kathryn Matt,&nbsp;Katharine S. Gries,&nbsp;Robin Carson,&nbsp;Fredrik Borgsten,&nbsp;Katja Weisel","doi":"10.1111/ejh.14392","DOIUrl":"10.1111/ejh.14392","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>This final post hoc analysis evaluated patient-reported outcomes from the Phase 3 MAIA study of daratumumab, lenalidomide, and dexamethasone (D-Rd) versus lenalidomide and dexamethasone (Rd) after median 64.5-month follow-up in transplant-ineligible patients with newly diagnosed multiple myeloma (NDMM), including patient subgroups.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Key scales from the EORTC QLQ-C30 (global health status [GHS], physical functioning, pain, and fatigue) were assessed. Scores were evaluated every 3 months for 1 year, then every 6 months until disease progression.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The intent-to-treat population (<i>n</i> = 737) included 46.3% frail, 35.4% 70 to &lt; 75 years old, and 43.6% ≥ 75 years old. D-Rd-treated patients showed improvements from baseline that were sustained over 5 years in the intent-to-treat population and across subgroups by age, frailty, and bone lesions. Greater proportions of patients treated with D-Rd versus Rd achieved minimally important changes for improvement at cycle 36 (year ~3) in GHS (odds ratio, 1.84 [95% CI, 1.16–2.91]), physical functioning (1.93 [1.18–3.14]), pain (1.41 [0.90–2.22]), and fatigue (2.00 [1.24–3.23]). Greater proportions of patients with bone lesions improved with D-Rd versus Rd on GHS and physical functioning.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>In transplant-ineligible patients with NDMM, D-Rd improved health-related quality of life over a 5-year period versus Rd.</p>\u0000 \u0000 <p>\u0000 <b>Trial Registration:</b> ClinicalTrials.gov: NCT02252172</p>\u0000 </section>\u0000 </div>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":"114 5","pages":"883-889"},"PeriodicalIF":2.3,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ejh.14392","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143425123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Atypical Hairy Cell Leukemia—The Current Status and Future Directions","authors":"Tadeusz Robak,&nbsp;Marta Robak,&nbsp;Agata Majchrzak,&nbsp;Anna Krawczyńska,&nbsp;Marcin Braun","doi":"10.1111/ejh.14388","DOIUrl":"10.1111/ejh.14388","url":null,"abstract":"<p>Hairy cell leukemia (HCL) is a rare, chronic lymphoid leukemia characterized by circulating lymphocytes with pale, hair-like cytoplasmic projections, pancytopenia, marked monocytopenia, and splenomegaly. Classic HCL displays distinct morphological, immunophenotypical, and genetic features. Classic HCL cells exhibit central nuclei, abundant cytoplasm with hair-like projections, and expression of CD20, CD22, CD11c, CD103, CD25, CD123, TBX21, annexin A1 (ANXA1), FMC7, CD200, and weak cyclin D1 (CCND1). While the vast majority of classic HCL cases harbor the <i>BRAF</i> V600E somatic mutation, rare examples have been reported without splenomegaly, with bulky lymphadenopathy, or with an atypical morphology, immunophenotype or genotype. This review analyzes the atypical clinical, morphologic, immunophenotypic, and genetic presentations associated with classic HCL. PubMed, Web of Science, and Google Scholar were searched for articles of hairy cell leukemia, including atypical morphology, atypical immunophenotype, atypical genotype, and rare symptoms. Publications from October 2004 to December 2024 were reviewed, with additional relevant studies obtained by reviewing references from selected articles.</p>","PeriodicalId":11955,"journal":{"name":"European Journal of Haematology","volume":"114 5","pages":"747-762"},"PeriodicalIF":2.3,"publicationDate":"2025-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ejh.14388","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143390564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}