Annals of Hematology最新文献

{"title":"Radiotherapy alone with curative intent in a case of limited-stage extranodal NK/T-cell lymphoma nasal type: a case report and review of the literature.","authors":"Biancamaria Mandelli, Donatella Caivano, Antonella Fontana, Natalia Cenfra, Sergio Mecarocci, Maristella Marrocco, David Fanciullo, Roberta Mazzarella, Martina Lorenzon, Giada Pacitto, Alessandro Pulsoni","doi":"10.1007/s00277-025-06260-x","DOIUrl":"https://doi.org/10.1007/s00277-025-06260-x","url":null,"abstract":"<p><p>Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT), is an aggressive malignancy primarily affecting the sinonasal region, with a strong association with Epstein-Barr virus (EBV) infection. The disease is significantly more prevalent in Asian and Latin American populations. Diagnosis is particularly challenging in nonendemic regions. We present the case of a 78-year-old male with a one-year history of nasal lesions, later diagnosed with ENKTCL-NT. The patient was treated with curative-intent radiotherapy, achieving a complete clinical response. Radiation therapy, particularly utilizing advanced techniques such as Volumetric Modulated Arc Therapy (VMAT), resulted in favorable outcomes with minimal toxicity. This case emphasizes the importance of early diagnosis, accurate staging, and personalized radiotherapy in the management of ENKTCL-NT. Ongoing research into the molecular pathogenesis, treatment strategies, and prognostic factors is crucial for improving outcomes, particularly in advanced-stage disease.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143771116","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":"Navigating the peginterferon Alfa-2a shortage: practical guidance on transitioning patients to ropeginterferon alfa-2b","authors":"Ruben Mesa,&nbsp;Abdulraheem Yacoub,&nbsp;Tsewang Tashi,&nbsp;Wanxing Chai-Ho,&nbsp;Chang Ho Yoon,&nbsp;John Mascarenhas","doi":"10.1007/s00277-025-06331-z","DOIUrl":"10.1007/s00277-025-06331-z","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 4","pages":"2571 - 2573"},"PeriodicalIF":3.0,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-025-06331-z.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143771115","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":"Real-world data of siltuximab for Chinese patients with iMCD: combination with BCD regimen as a potential approach for severe cases","authors":"Si-yuan Li,&nbsp;Yu-han Gao,&nbsp;Yue Dang,&nbsp;Long Chang,&nbsp;Kai-ni Shen,&nbsp;Hua-cong Cai,&nbsp;Dan-qing Zhao,&nbsp;Chong Wei,&nbsp;Jun Feng,&nbsp;Lu Zhang,&nbsp;Jian Li","doi":"10.1007/s00277-025-06329-7","DOIUrl":"10.1007/s00277-025-06329-7","url":null,"abstract":"<div><p>Idiopathic multicentric Castleman disease (iMCD) is a rare disease characterized by polyclonal lymphoproliferation and systemic inflammation. Siltuximab, targeting interleukin-6 (IL-6), has been recommended as the first-line therapy for iMCD. However, substantial real-world data from China were still lacking, and treatment for patients with severe iMCD remained challenging. This single-center retrospective study investigated the real-world efficacy and safety of siltuximab-based therapy in 43 consecutive patients with iMCD in China from July 2022 to March 2024. The overall response rate (including symptomatic and biochemical response) was 59% at week 3 and increased to 91% at week 12, with complete and partial response rates of 54% and 37%, respectively. Patients who received siltuximab as a first-line treatment exhibited better treatment response (OR = 0·040, 95% CI, 0·004 − 0·390, <i>p</i> = 0·006). Inflammatory markers (such as interleukin-6 and high-sensitivity C-reactive protein [hsCRP]) and pathologic types showed no predictive role in the treatment responses. Eighteen patients, who were all classified as severe iMCD, received combined therapy of siltuximab and BCD regimen (bortezomib, cyclophosphamide and dexamethasone). Of them, the overall response rate was 50% at week 3, which increased to 100% at week 12. Our findings reinforced the existing evidence on the efficacy and safety of siltuximab and highlighted the potential benefits of combining siltuximab with BCD regimen in severe iMCD patients.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 3","pages":"1713 - 1720"},"PeriodicalIF":3.0,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-025-06329-7.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143771119","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":"Building a new score system for the diagnosis and differential diagnosis of typical CLL/SLL, atypical CLL/SLL, and MCL based on the flow cytometry immunophenotyping","authors":"Emile Niyonizeye,&nbsp;Xiaoqian Wang,&nbsp;Dongyao Yan,&nbsp;Seidu A. Richard,&nbsp;Huijun Ren,&nbsp;Liyan Fu,&nbsp;Yaoyao Chen,&nbsp;Xiaogeng Yuan,&nbsp;Baohong Yue","doi":"10.1007/s00277-025-06231-2","DOIUrl":"10.1007/s00277-025-06231-2","url":null,"abstract":"<div><p>The diagnosis and differential diagnosis of typical lymphocytic leukaemia (CLL)/Small lymphocytic lymphoma(SLL) especially atypical CLL/SLL (aCLL/SLL), and mantle cell lymphoma (MCL) remains highly challenging. Thus, we evaluated the accuracy of the combination of the Moreau score system (MSS) indicators (markers), with three new indicators CD19/CD20 ratio, CD200, and CD43 in diagnosis and differential diagnosis of typical CLL/SLL, atypical CLL/SLL, and MCL. We retrospectively retrieved data from peripheral blood, bone marrow, and lymph nodes of 120 patients comprising 64 typical CLL, 10 atypical CLL, and 46 MCL from March 2020 to September 2023 at the First Affiliated Hospital of Zhengzhou University. Multiparametric evaluation flow cytometry and immunophenotype were obtained via a B-cell panel and pathological reports were carried out. We observe that the new score system (NSS) and the MSS had the same specificity. However, the NSS demonstrated to have superior sensitivity compared to MSS. Adding three new markers CD19/CD20, CD200, and CD43 is accountable for the superior sensitivity in our NSS. Also, a combination of markers such as CD19, CD20, CD5, CD23, CD79b, FMC7, SmIg, CD22, CD43, CD200, the ratio CD19/CD20 may be a potential differentiating modality for typical CLL/SLL, atypical CLL/SLL, and MCL. Adding the CD19/CD20 ratio, CD43, and CD200 indicators to the MSS indicators could be potentially accurate diagnostic and differential diagnosis modality for typical CLL/SLL, atypical CLL/SLL, and MCL.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 3","pages":"1807 - 1819"},"PeriodicalIF":3.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-025-06231-2.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143750832","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":"Contribution of molecular characterization to the diagnosis of MPN in patients with low JAK2V617F variant allelic fraction in a real-world cohort","authors":"Benjamin Lebecque,&nbsp;Carolyne Croizier,&nbsp;Thomas Tassin,&nbsp;Esteban Louis,&nbsp;Nathalie Tribalat,&nbsp;William Bombardier,&nbsp;Anne-Pascale Grandjean,&nbsp;Vanessa Pante,&nbsp;Hélène Monjanel,&nbsp;Jean-Baptiste Bouillon-Minois,&nbsp;Rémi Martel,&nbsp;Albane Ledoux-Pilon,&nbsp;Nathalie Boiret-Dupré,&nbsp;Benoit De Renzis,&nbsp;Marc Gabriel Berger,&nbsp;Céline Bourgne","doi":"10.1007/s00277-025-06326-w","DOIUrl":"10.1007/s00277-025-06326-w","url":null,"abstract":"<div><p>Since 2008, the <i>JAK2</i>^<i>V617F</i> mutation has been key for diagnosing myeloproliferative neoplasms (MPN) according to the World Health Organization criteria. However, the clinical and biological significance of low <i>JAK2</i>^<i>V617F</i> variant allelic fraction (VAF) remains poorly understood. To address this, we performed a comprehensive molecular characterization of a monocentric real-world retrospective cohort of MPN patients with low JAK2^V617F VAF, diagnosed between 2007 and 2019. Our analysis revealed that 46.3% of these cases had additional driver mutations into <i>JAK2</i>, <i>CALR</i>, and <i>MPL</i> genes associated with very low <i>JAK2</i>^V617F VAF (median: 0.09%). Furthermore, next-generation sequencing of cases without these driver mutations showed that 67.7% harbored other mutations, including low VAF <i>CALR</i> mutations, as well as <i>TP53</i> alterations or predisposition genes. These findings highlight the importance of comprehensive molecular analysis in conjunction with bone marrow biopsy (BMB). Notably, we found a negative BMB did not exclude an MPN diagnosis, and molecular results confirmed MPN in some patients even without BMB evidence. Integrating BMB findings, molecular data, and low <i>JAK2</i>^V617F VAF with clinical assessments highlights the potential for misdiagnoses, especially in cases that might overlap with age-related clonal hematopoiesis. Our study emphasizes the need for extensive molecular investigation in cases of low <i>JAK2</i>^V617F MPN to ensure accurate diagnosis and appropriate management.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 3","pages":"1587 - 1596"},"PeriodicalIF":3.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-025-06326-w.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143750754","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":"Correction to: Evolution of frontline treatment for multiple myeloma: clinical investigation of quadruplets containing carflzomib and antiCD38 monoclonal antibodies","authors":"Luciano J. Costa,&nbsp;Francesca Gay,&nbsp;Ola Landgren,&nbsp;María-Victoria Mateos,&nbsp;Philippe Moreau,&nbsp;Cyrille Touzeau,&nbsp;Franziska Ertel,&nbsp;Ian McFadden,&nbsp;Rani Najdi,&nbsp;Katja Weisel","doi":"10.1007/s00277-025-06333-x","DOIUrl":"10.1007/s00277-025-06333-x","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 3","pages":"1353 - 1353"},"PeriodicalIF":3.0,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-025-06333-x.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143750758","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":"Comparative efficacy of venetoclax-based regiments in the management of chronic lymphocytic leukemia: a systematic review and metanalysis","authors":"Abdur Jamil,&nbsp;Shehroz Aslam,&nbsp;Rida Riaz,&nbsp;Zaheer Qureshi,&nbsp;Hamzah Akram,&nbsp;Asim Kichloo,&nbsp;Insija Ilyas Selene","doi":"10.1007/s00277-025-06280-7","DOIUrl":"10.1007/s00277-025-06280-7","url":null,"abstract":"<div><p>Chronic lymphocytic leukemia and small lymphocytic lymphoma (CLL/SLL) are common lymphoproliferative diseases in the elderly, accounting for 33% of all leukemias. The use of targeted therapies has significantly changed the management landscape of CLL/SLL. However, the efficacy of specific targeted therapies, such as venetoclax-based therapies, is limited. This review, therefore, aims to summarize the current evidence on the effectiveness and safety of venetoclax-based treatments in the management of CLL/SLL. We conducted a comprehensive search of three electronic databases, PubMed, Science Direct and Google Scholar, for all relevant articles. The reported outcomes were then analyzed using the statistical software Review Manager (RevMan 5.4.1). Our electronic search yielded 347 articles, of which only five were included in the review. We pooled the outcomes from 2195 patients. Our analysis found that venetoclax-based therapies significantly increased progression-free survival (PFS) (HR 0.30; 95% CI [0.21, 0.43] <i>p</i> &lt; 0.00001), overall survival (OS) (HR 0.60; 95% CI [0.45, 0.80] <i>p</i> = 0.0004), and time to the next treatment (TTNT) (HR 0.29; 95% [0.18, 0.46] <i>p</i> &lt; 0.00001). Additionally, we found the comparative safety of venetoclax-based therapies to other combination therapies. Our study found that venetoclax-based therapies are superior to other combination therapies in prolonging survival. Furthermore, they are comparable in safety to standard chemotherapy regimens and cost-effective. The findings of this review provide preliminary evidence on the efficacy of venetoclax-based regimens in CLL/SLL. However, further research is required to determine the best comparative regimen and the feasibility of venetoclax monotherapy in patients with CLL/SLL.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 3","pages":"1387 - 1397"},"PeriodicalIF":3.0,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-025-06280-7.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143742010","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":"A phase 1 study of durvalumab as monotherapy or combined with tremelimumab with or without azacitidine in patients with myelodysplastic syndrome","authors":"Guillermo Garcia-Manero,&nbsp;Manila Gaddh,&nbsp;Uwe Platzbecker,&nbsp;R. Coleman Lindsley,&nbsp;Sarah M. Larson,&nbsp;Timothy Chevassut,&nbsp;Pierre Fenaux,&nbsp;Rami Komrokji,&nbsp;Roger Lyons,&nbsp;Aref Al-Kali,&nbsp;Yu Jiang,&nbsp;John Bothos,&nbsp;Danielle M. Townsley,&nbsp;Amer M. Zeidan","doi":"10.1007/s00277-024-06081-4","DOIUrl":"10.1007/s00277-024-06081-4","url":null,"abstract":"<div><p>Upregulation of programmed death ligand-1 (PD-L1) has been observed in patients with MDS, and its expression on myeloblasts is associated with progression to AML. This open-label, phase 1 study evaluated the safety and tolerability of the PD-L1 antibody durvalumab as monotherapy (part 1) and in combination with tremelimumab, with or without azacitidine (part 2), in patients with MDS who progressed following hypomethylating agent treatment. Sixty-seven adults with MDS were enrolled (part 1, 40 with low/intermediate-1 or intermediate-2/high IPSS risk status; part 2, 27 with intermediate-2/high IPSS risk status). Primary safety endpoints included dose-limiting toxicities (DLTs) and treatment-emergent adverse events (TEAEs). Secondary endpoints included evaluation of clinical outcomes, survival, and pharmacokinetics. Dose-limiting toxicities were experienced by no patients in part 1 and 3 patients (11%) in part 2. The most common treatment-emergent adverse events were diarrhea and fatigue (40% each) in part 1 and fatigue (44%) and anemia (37%) in part 2. In parts 1 and 2, 15% of patients experienced marrow complete response as their best overall response, according to IWG criteria. Hematologic improvement was observed in 35% and 30% of patients respectively in part 1 and part 2. The study was terminated early due to limited efficacy.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 3","pages":"1577 - 1585"},"PeriodicalIF":3.0,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-024-06081-4.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143735588","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":"First-line treatment of TAFRO syndrome with rituximab: a case report and literature review.","authors":"Salar Servati, Ida Mohammadi, Shahryar Rajai Firouzabadi","doi":"10.1007/s00277-025-06327-9","DOIUrl":"https://doi.org/10.1007/s00277-025-06327-9","url":null,"abstract":"<p><p>TAFRO syndrome, a rare variant of Castleman's disease, is characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. First-line treatments typically include immunosuppressive therapies such as cyclosporine A and tocilizumab. However, the use of rituximab as a first-line therapeutic agent remains under-reported. Here, we present the case of a 38-year-old female patient who initially exhibited lymphadenopathy and gradually developed the full spectrum of symptoms associated with TAFRO syndrome during her hospital stay. Rituximab, supplemented by prednisolone, was administered as the primary treatment. Over a follow-up period of five months, the patient's condition improved significantly, suggesting a positive therapeutic response. This case highlights the potential efficacy of rituximab as an alternative first-line therapy for TAFRO syndrome, offering a possible treatment option where standard therapeutic agents may not be available, suitable or effective.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143727621","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":"Spontaneous remission of iMCD-TAFRO: a case report","authors":"Yuchong Qiu,&nbsp;Miaoyan Zhang,&nbsp;Congwei Jia,&nbsp;Lu Zhang,&nbsp;Jian Li","doi":"10.1007/s00277-025-06324-y","DOIUrl":"10.1007/s00277-025-06324-y","url":null,"abstract":"<div><p>Idiopathic multicentric Castleman disease (iMCD)- TAFRO (thrombocytopenia, ascites, fever, reticulin fibrosis and organomegaly) is a clinically severe situation characterized by cytokine storms that are potentially fatal, necessitating prompt and decisive medical intervention. The International Castleman’s Disease Collaborative Network (CDCN) prioritizes the combination of long-term siltuximab and high-dose steroids as the preferred therapeutic option for iMCD-TAFRO. Here, we report a rare case of spontaneous remission in iMCD-TAFRO. In this case, the patient diagnosed with iMCD-TAFRO experienced alleviation of clinical symptoms and normalization of laboratory test results without undergoing any etiological treatment. This case sheds new light on the therapeutic strategies for iMCD-TAFRO. Following the stabilization of the onset cytokine storm, a detailed evaluation of the patient’s condition should be performed to determine the need for continuous medical treatment.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"104 4","pages":"2559 - 2561"},"PeriodicalIF":3.0,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-025-06324-y.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143727656","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}