Annals of Hematology最新文献_第3页

Contribution of molecular characterization to the diagnosis of MPN in patients with low JAK2^V617F variant allelic fraction in a real-world cohort.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-04-01 DOI: 10.1007/s00277-025-06326-w

Benjamin Lebecque, Carolyne Croizier, Thomas Tassin, Esteban Louis, Nathalie Tribalat, William Bombardier, Anne-Pascale Grandjean, Vanessa Pante, Hélène Monjanel, Jean-Baptiste Bouillon-Minois, Rémi Martel, Albane Ledoux-Pilon, Nathalie Boiret-Dupré, Benoit De Renzis, Marc Gabriel Berger, Céline Bourgne

{"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, Carolyne Croizier, Thomas Tassin, Esteban Louis, Nathalie Tribalat, William Bombardier, Anne-Pascale Grandjean, Vanessa Pante, Hélène Monjanel, Jean-Baptiste Bouillon-Minois, Rémi Martel, Albane Ledoux-Pilon, Nathalie Boiret-Dupré, Benoit De Renzis, Marc Gabriel Berger, Céline Bourgne","doi":"10.1007/s00277-025-06326-w","DOIUrl":"https://doi.org/10.1007/s00277-025-06326-w","url":null,"abstract":"Since 2008, the JAK2V617F mutation has been key for diagnosing myeloproliferative neoplasms (MPN) according to the World Health Organization criteria. However, the clinical and biological significance of low JAK2V617F 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 JAK2V617F VAF, diagnosed between 2007 and 2019. Our analysis revealed that 46.3% of these cases had additional driver mutations into JAK2, CALR, and MPL genes associated with very low JAK2V617F VAF (median: 0.09%). Furthermore, next-generation sequencing of cases without these driver mutations showed that 67.7% harbored other mutations, including low VAF CALR mutations, as well as TP53 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 JAK2V617F 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 JAK2V617F MPN to ensure accurate diagnosis and appropriate management.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Correction to: Evolution of frontline treatment for multiple myeloma: clinical investigation of quadruplets containing carflzomib and antiCD38 monoclonal antibodies.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-31 DOI: 10.1007/s00277-025-06333-x

Luciano J Costa, Francesca Gay, Ola Landgren, María-Victoria Mateos, Philippe Moreau, Cyrille Touzeau, Franziska Ertel, Ian McFadden, Rani Najdi, Katja Weisel

引用次数: 0

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-31 DOI: 10.1007/s00277-025-06310-4

Ran An, Weiying Bao, Zeying Yan, Haimin Sun, Xiaoqian Xu, Sujiang Zhang

引用次数: 0

Comparative efficacy of venetoclax-based regiments in the management of chronic lymphocytic leukemia: a systematic review and metanalysis.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-29 DOI: 10.1007/s00277-025-06280-7

Abdur Jamil, Shehroz Aslam, Rida Riaz, Zaheer Qureshi, Hamzah Akram, Asim Kichloo, Insija Ilyas Selene

{"title":"Comparative efficacy of venetoclax-based regiments in the management of chronic lymphocytic leukemia: a systematic review and metanalysis.","authors":"Abdur Jamil, Shehroz Aslam, Rida Riaz, Zaheer Qureshi, Hamzah Akram, Asim Kichloo, Insija Ilyas Selene","doi":"10.1007/s00277-025-06280-7","DOIUrl":"https://doi.org/10.1007/s00277-025-06280-7","url":null,"abstract":"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] p < 0.00001), overall survival (OS) (HR 0.60; 95% CI [0.45, 0.80] p = 0.0004), and time to the next treatment (TTNT) (HR 0.29; 95% [0.18, 0.46] p < 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.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A phase 1 study of durvalumab as monotherapy or combined with tremelimumab with or without azacitidine in patients with myelodysplastic syndrome.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-28 DOI: 10.1007/s00277-024-06081-4

Guillermo Garcia-Manero, Manila Gaddh, Uwe Platzbecker, R Coleman Lindsley, Sarah M Larson, Timothy Chevassut, Pierre Fenaux, Rami Komrokji, Roger Lyons, Aref Al-Kali, Yu Jiang, John Bothos, Danielle M Townsley, Amer M Zeidan

{"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, Manila Gaddh, Uwe Platzbecker, R Coleman Lindsley, Sarah M Larson, Timothy Chevassut, Pierre Fenaux, Rami Komrokji, Roger Lyons, Aref Al-Kali, Yu Jiang, John Bothos, Danielle M Townsley, Amer M Zeidan","doi":"10.1007/s00277-024-06081-4","DOIUrl":"https://doi.org/10.1007/s00277-024-06081-4","url":null,"abstract":"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.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

First-line treatment of TAFRO syndrome with rituximab: a case report and literature review.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-26 DOI: 10.1007/s00277-025-06327-9

Salar Servati, Ida Mohammadi, Shahryar Rajai Firouzabadi

引用次数: 0

Spontaneous remission of iMCD-TAFRO: a case report.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-26 DOI: 10.1007/s00277-025-06324-y

Yuchong Qiu, Miaoyan Zhang, Congwei Jia, Lu Zhang, Jian Li

引用次数: 0

Low levels of CD3 + and CD8 + T cells in peripheral blood can predict poor efficacy of first-line chemotherapy in patients with angioimmunoblastic T cell lymphoma.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-26 DOI: 10.1007/s00277-025-06314-0

Yanfei Liu, Weiwei Song, Yuqin Song, Jun Zhu, Yan Xie

{"title":"Low levels of CD3 + and CD8 + T cells in peripheral blood can predict poor efficacy of first-line chemotherapy in patients with angioimmunoblastic T cell lymphoma.","authors":"Yanfei Liu, Weiwei Song, Yuqin Song, Jun Zhu, Yan Xie","doi":"10.1007/s00277-025-06314-0","DOIUrl":"https://doi.org/10.1007/s00277-025-06314-0","url":null,"abstract":"To investigate the prognostic significance of lymphocyte subsets in newly diagnosed angioimmunoblastic T-cell lymphoma (AITL) patients and track the dynamic changes in these subsets during treatment. We retrospectively analyzed the medical records in 56 patients with AITL. Lymphocyte subsets were assessed by flow cytometry at diagnosis. Receiver operating characteristic curves were employed to establish optimal cut-off values for lymphocyte subset assessment. Univariate and multivariate analyses were used to evaluate overall survival (OS) and progression-free survival (PFS). The first-line treatment demonstrated an overall response rate (ORR) of 71.4%. Optimal cut-off values for ORR were determined for CD3 + T cell percentage (61.9%) and CD8 + T cell percentage (28.4%), with corresponding AUC of 0.756 [95% CI (0.611-0.902), p = 0.003] and 0.745 [95% CI (0.596-0.894), p = 0.004]. The ORR for the Low CD3+/CD8 + Group (CD3 + T < 61.9% and CD8 + T < 28.4%) and High CD3+/CD8 + Group (CD3 + T ≥ 61.9% or CD8 + T ≥ 28.4%) was 25.0% and 84.1%, respectively (p < 0.001). With a median follow-up time of 37.4 months, the median PFS were 5.6 and 16.0 months for the both groups. After two cycles of chemotherapy, a significant elevation in the percentage of CD3 + T cells was observed comparing to pre-treatment. Three months post-treatment, the CD4/CD8 ratio reduced significantly, indicating a rebalancing of T cell populations. Reduced levels of CD3 + and CD8 + T lymphocyte may indicate inferior chemotherapy efficacy and poorer PFS in AITL. Monitoring the baseline lymphocyte subsets and their dynamic changes can assist in clinical treatment decision - making.","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":"143727638","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}

引用次数: 0

Monocytic meningitis complicating histiocytosis and response to MEK-inhibitor: a case series.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-25 DOI: 10.1007/s00277-025-06317-x

Tom Abrassart, Ahmed Idbaih, Damien Roos-Weil, Damien Galanaud, Fleur Cohen-Aubart, Jean-François Emile, Pierre Boncoeur, Zahir Amoura, Danielle Seilhean, Julien Haroche, Matthias Papo

{"title":"Monocytic meningitis complicating histiocytosis and response to MEK-inhibitor: a case series.","authors":"Tom Abrassart, Ahmed Idbaih, Damien Roos-Weil, Damien Galanaud, Fleur Cohen-Aubart, Jean-François Emile, Pierre Boncoeur, Zahir Amoura, Danielle Seilhean, Julien Haroche, Matthias Papo","doi":"10.1007/s00277-025-06317-x","DOIUrl":"https://doi.org/10.1007/s00277-025-06317-x","url":null,"abstract":"Central nervous system (CNS) involvement is common in histiocytosis, yet cerebrospinal fluid (CSF) analysis often yields normal results. We present three cases of monocytic meningitis associated with histiocytosis. The first patient was diagnosed with Erdheim-Chester disease (ECD) and exhibited evidence of a MAP2K1 mutation, concomitant with chronic myelomonocytic leukemia. Brain magnetic resonance imaging (MRI) revealed leptomeningitis and pachymeningitis. The presence of the same MAP2K1 mutation in CSF monocytes confirmed the clonal origin of neuromeningeal infiltration. Treatment with binimetinib rapidly improved the patient's clinical condition. The second case involved CNS primary malignant histiocytosis (CNS-PMH) associated with myelodysplastic syndrome. However, treatment with binimetinib only led to a partial and time-limited response. The last patient was diagnosed with mixed histiocytosis ECD/Rosai-Dorfman disease (RDD). Cobimetinib also proved effective in managing CNS symptoms. CSF pleocytosis in CNS involvement of histiocytosis has been reported in a few published cases with neurological involvement. Given its rarity, the presence of monocytic meningitis should prompt immediate suspicion of histiocytosis, particularly if accompanied by typical manifestations. In cases of neurological involvement in histiocytosis, lumbar puncture and liquid biopsy can sometime overcome the need for a meningeal biopsy. The molecular characterization of histiocytosis is essential for considering the use of targeted therapy, but the lack of an identified mutation should not preclude the use of anti-MEK therapy.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143708113","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}

引用次数: 0

Advances in classification and treatment of primary cutaneous lymphomas.

IF 3 3区医学

Annals of Hematology Pub Date : 2025-03-25 DOI: 10.1007/s00277-025-06311-3

Hong Zheng, Lihua Qiu, Chang Liu, Chen Tian

引用次数: 0