Annals of Hematology最新文献

{"title":"Secondary neoplasms in mycosis fungoides: evaluating risk factors and phototherapy impact in a comparative study.","authors":"Ayda Acar, Ayris Ozturk, Gunseli Ozturk, Ilgen Ertam Sagduyu, Bengu Gerceker Turk, Banu Yaman, Taner Akalın","doi":"10.1007/s00277-025-06442-7","DOIUrl":"https://doi.org/10.1007/s00277-025-06442-7","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144309469","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":"IGH/IGK gene rearrangement in the diagnosis of B-cell non-Hodgkin lymphoma: experience from three centers.","authors":"Ke Yang, Zhizhong Wang, Beibei Xin, Yunhang Li, Jiuzhou Zhao, Rui Sun, Weizhen Wang, Dongxu Chen, Chengzhi Zhao, Yongjun Guo, Jie Ma, Bing Wei","doi":"10.1007/s00277-025-06452-5","DOIUrl":"https://doi.org/10.1007/s00277-025-06452-5","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293243","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":"Good response to oxymetholone in adult aplastic anemia.","authors":"Jindaratn Chaipokam, Ponlapat Rojnuckarin","doi":"10.1007/s00277-025-06460-5","DOIUrl":"https://doi.org/10.1007/s00277-025-06460-5","url":null,"abstract":"<p><p>In Thailand, stem cell transplantation and horse antithymocyte globulin (ATG) are not accessible for most adult aplastic anemia (AA) patients. Alternative therapies are required. We conducted a cohort study of 110 adult AA patients treated with oxymetholone alone for at least 30 days from 2013 to 2023. Response at month 6 and prognostic factors were evaluated. The mean age was 63.4 years old and 58.2% were female. Severe and very severe AA (SAA/VSAA) comprised 64.5% and 3.6%, respectively. The initial oxymetholone daily dose was 150 mg in 66.4%. The overall response was 56.4% (50.7% for SAA/VSAA), with a median time to transfusion independence of 11.8 weeks. Deaths were regarded as no response. Seventeen (17.9%) patients discontinued the treatment due to side effects, especially hepatitis (15/17). Androgenic side effects (55.5%) mostly occurred within the first month. Multivariate analysis identified that baseline reticulocyte count > 10 × 10⁹/L (adjusted odds ratio [OR] 7.3, 95% confidence interval [CI] (2.55-21.11), oily skin (OR 4.93, 95%CI 1.50-16.26) and acne (OR 9.78, 95%CI 2.11-45.28) occurring within 2 months were predictive for responses. The SKAR scoring system using these three factors showed an area under the ROC curve of 0.87 (95%CI 0.80-0.92). The 5-year overall survival rate was 77.4%. Poor performance status (p < 0.001) and response status (p < 0.001) significantly impacted mortality. Responding patients demonstrated 94.5% 5-year survival. In conclusion, androgen is a useful treatment option for AA in Thailand. The score based on reticulocytes and androgenic effects could predict the response and potentially help decision-making.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293242","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":"Autologous stem cell transplantation with thiotepa, busulfan, and cyclophosphamide conditioning in patients with central nervous system lymphoma: a phase II study.","authors":"Dong Hyun Kim, Taekeun Park, Junshik Hong, Dong-Yeop Shin, Inho Kim, Sung-Soo Yoon, Ja Min Byun, Youngil Koh","doi":"10.1007/s00277-025-06405-y","DOIUrl":"https://doi.org/10.1007/s00277-025-06405-y","url":null,"abstract":"<p><p>The prognosis of central nervous system lymphoma (CNSL) remains poor, and attempts have been made to improve outcomes through autologous stem cell transplantation (ASCT) with thiotepa-based conditioning. We aimed to assess the outcomes of thiotepa/busulfan/cyclophosphamide (TBC) followed by ASCT in CNSL. An investigator-initiated, single-arm, phase II trial was conducted to evaluate the efficacy and safety of TBC/ASCT (NCT06625359). The conditioning dose was adjusted based on age and performance status (9-day or 8-day regimen). As this trial was terminated early after enrolling 17 patients, a retrospective cohort of CNSL treated with the same protocol was included. In total, 44 patients were included in the study and classified into 8-day and 9-day groups according to the TBC regimen received. In total, 25 patients (56.8%) had primary CNSL, and 19 patients (43.2%) received the 9-day regimens. Following ASCT, 33 patients (75.0%) achieved an objective response (32 complete response and 1 partial response). The 3-year progression-free survival (46.5% vs. 52.6%, P = 0.49) and overall survival (60.5% vs. 73.7%, P = 0.77) for 8-day and 9-day groups were comparable. The 1-year cumulative incidence of non-relapse mortality in the 8-day group showed a trend toward decrease compared to that in the 9-day group (4.6% vs. 21.1%, P = 0.073). Our study demonstrates the efficacy of TBC/ASCT in CNSL in the Asian population. The conventional busulfan dose (9-day regimen) may be associated with higher toxicity, suggesting the potential need for a modified TBC regimen in Asian populations. Further studies are needed to identify the optimal thiotepa-based conditioning in CNSL.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293239","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":"Efficacy and safety of Glofitamab in patients with R/R DLBCL in real life setting- a retrospective study.","authors":"Ronit Gurion, Dmitri Guz, Meirav Kedmi, Chava Perry, Irit Avivi, Netanel A Horowitz, Uri Abadi, Anat Gafter-Gvili, Pia Raanani, Neta Goldschmidt, Boaz Nachmias, Shlomzion Aumann","doi":"10.1007/s00277-025-06438-3","DOIUrl":"https://doi.org/10.1007/s00277-025-06438-3","url":null,"abstract":"<p><p>Glofitamab, a CD20-directed CD3 T-cell engager, was recently FDA-approved after demonstrating a 52% overall response rate (ORR) and a 39% complete response (CR) rate in heavily pretreated diffuse large B-cell lymphoma (DLBCL) patients. However, real-world data on its efficacy and safety remain limited. This study evaluated glofitamab's performance in clinical practice. We conducted a retrospective multicenter study of adults with relapsed/refractory (R/R) DLBCL treated via a national compassionate use program. Patients received at least one dose of glofitamab after failing ≥ 2 prior therapies. Recruitment spanned September 2020-January 2023. Outcomes included ORR, CR (per Lugano criteria), progression-free survival (PFS), and overall survival (OS). Adverse events were classified per ASTCT 2019 criteria, and risk factors for PFS and OS were assessed via logistic regression. Thirty-five patients from six Israeli centers were included (median age: 67 years; 66% male). The median number of prior therapies was 5, with 43% being primary refractory and 91% post-CAR-T therapy. ORR was 34%, with 14% achieving CR. Median PFS and OS were 2 and 4 months, respectively. Treatment was prematurely discontinued in 86%, mainly due to disease progression (46%) and to infections in responding patients (17%). Male sex was a significant risk factor for poor PFS and OS. In this real-world cohort, glofitamab's efficacy was lower than in clinical trials, likely due to a more heavily pretreated population. However, its manageable toxicity supports its potential role in r/r DLBCL treatment.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144282142","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":"High-dose romiplostim for donor-type aplasia.","authors":"Kohei Hosokawa, Tatsuya Imi, Takafumi Yokota, Yuma Tada, Hiroyuki Maruyama, Naomi Sugimori, Ken Ishiyama, Hirohito Yamazaki, Toshihiro Miyamoto, Shinji Nakao","doi":"10.1007/s00277-025-06448-1","DOIUrl":"https://doi.org/10.1007/s00277-025-06448-1","url":null,"abstract":"","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144274051","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":"Feasibility analysis of metabolic parameters based on baseline ¹⁸F-FDG PET/CT to predict heterogeneity and recurrence of diffuse large B-cell lymphoma.","authors":"Fan Ge, Tingting Wu, Xinyue Yang, Mengye Peng, Chen Yang, Kezheng Wang","doi":"10.1007/s00277-025-06409-8","DOIUrl":"https://doi.org/10.1007/s00277-025-06409-8","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to evaluate the predictive value of intra-tumoral ¹⁸F-FDG metabolic heterogeneity in patients with diffuse large B cell lymphoma (DLBCL) in terms of survival.</p><p><strong>Methods: </strong>We retrospectively included 245 patients with DLBCL who underwent ¹⁸F-FDG PET/CT prior to treatment and analyzed using total metabolic tumor volume (TMTV) and total lesion glycolysis (TLG) as metabolic volume parameters. The linear regression slopes of TMTV and TLG were calculated according to different percentages of SUV thresholds (i.e., 40%, 50%, 60%, 70%, and 80%), respectively, defined as Heterogeneity Factor-1 (HF1) and Heterogeneity Factor-2 (HF2). These indices of heterogeneity were used to predict progression-free survival (PFS). Based on the results of the Cox proportional hazards model, we constructed a multi-parameter prediction model and evaluated the model in the training and validation cohorts by calibration curve, consistency index (C-index) and decision curve analysis (DCA).</p><p><strong>Results: </strong>Clinicopathological and PET/CT data from 245 patients were reviewed. 153 patients (62.4%) experienced relapse after treatment. Comparing relapsed and non-relapse patients, all ¹⁸F-FDG PET/CT parameters and heterogeneity index showed significant differences. There were significant differences in survival risk stratification according to HF1 and HF2 cut-off classifications (P < 0.0001). In multivariate Cox regression analysis, SUVmax (P < 0.0001), TLG (P < 0.0001), HF1 (P = 0.004), and HF2 (P < 0.0001) showed significant results. Among the clinicopathological parameters, IPI (P = 0.027) and Size (P < 0.0001) were selected as important parameters.</p><p><strong>Conclusions: </strong>HF1 and HF2 obtained by the linear regression slope of MTV and TLG may be a novel and useful prognostic marker in DLBCL, which can achieve survival-risk stratification of patients. In addition, multiparametric models have the potential to effectively predict the risk of recurrence in patients.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144265156","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":"Assessing serum thrombopoietin for enhanced diagnosis of ITP, AA, and MDS using machine learning: A retrospective cohort study.","authors":"Guoqing Zhu, Yansong Ren, Lele Wang, Shoulei Wang, Yansheng Wang, Yulong Fan, Lunhui Huang, Yonghui Xia, Liwei Fang","doi":"10.1007/s00277-025-06308-y","DOIUrl":"https://doi.org/10.1007/s00277-025-06308-y","url":null,"abstract":"<p><p>Differentiating between immune thrombocytopenia (ITP), aplastic anemia (AA), and myelodysplastic syndromes (MDS) is critical due to the distinct treatment approaches required for each condition. This study investigates the role of serum thrombopoietin (TPO) levels as a potential biomarker to aid in the diagnosis of these hematological disorders. This retrospective cohort study analyzed serum TPO levels in patients diagnosed with ITP, AA, and MDS, using clinical records and stored serum samples collected from patients treated between September 2023 and May 2024. Statistical analyses were performed to determine cut-off values for TPO levels that effectively differentiate between these conditions. Additionally, machine learning models were utilized to enhance diagnostic accuracy based on clinical indicators, including TPO levels. Serum TPO levels were markedly elevated in AA (1369.19 ± 751.26 pg/ml) compared to ITP (263.57 ± 355.91 pg/ml), MDS (434.55 ± 551.56 pg/ml), and health control (71.64 ± 30.32 pg/ml) (P < 0.0001). Correlation analysis revealed a significant positive correlation between TPO levels and ITP, AA, and MDS (P < 0.0001), Linear regression analysis indicated that age was a significant predictor of TPO levels (P < 0.0001). The optimal cut-off value for TPO levels distinguishing ITP from AA was 302.43 pg/mL, yielding an AUC of 0.925 (sensitivity with 80.75%, specificity with 94.06%). Machine learning models demonstrated that Logistic Regression, XGBoost, and LightGBM performed best, with the Logistic Regression achieving an accuracy of 86.3% and an AUC of 0.910. Serum TPO levels are a promising non-invasive biomarker for distinguishing between ITP, AA, and MDS. Incorporating TPO measurements into clinical practice may enhance diagnostic accuracy and improve patient management strategies.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144257246","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":"Ixazomib-lenalidomid-dexamethasone (IRd) in relapsed refractory multiple myeloma (RRMM)-multicenter real-world analysis from Germany and comparative review of the literature.","authors":"Hanna Löffler, Magdalena Braun, Heike Reinhardt, Amelie Rösner, Gabriele Ihorst, Jan Räder, Sina Wenger, Annamaria Brioli, Marie von Lilienfeld-Toal, Maika Klaiber-Hakimi, Eva-Marie Fehr, Markus Munder, Katharina Epp, Karolin Trautmann-Grill, Sarah Decker, Holger Hoff, Ralph Wäsch, Monika Engelhardt","doi":"10.1007/s00277-025-06441-8","DOIUrl":"https://doi.org/10.1007/s00277-025-06441-8","url":null,"abstract":"<p><p>With the introduction of novel agents (proteasome inhibitors (PIs), immunomodulatory drugs (IMiDs), immuntherapeutics), the outcome of multiple myeloma (MM) patients has improved. The oral PI ixazomib was authorized in the EU in 11/2016 and is indicated in combination with lenalidomide and dexamethasone (IRd) for the treatment of patients with relapsed/refractory MM (RRMM). The study aim was to identify the typical real world (RW) RRMM patient population in Germany treated with IRd and to compare the outcome to prior IRd cohorts. Our retrospective study included consecutive RRMM patients receiving IRd in six large German hospitals between 06/2017 and 12/2021. We identified 24 consecutive RRMM patients within these six institutions. Their median age was 68 years at the start of IRd; 21% carrying high-risk cytogenetics [del17p, t(4;14) and/or t(14;16)]. Patients had a median of 2 prior treatment lines (range, 1-5), prior PI/IMiD exposure was 100%/58%, comparable to previous real world evidence (RWE)-studies. With a median follow-up of 37.7 months, the overall response rate was 70.8%, and median PFS and OS were 22.0 and 62.2 months, respectively. With these German data, our study is closing an important gap of IRd real world data (RWD) reporting across Europe, providing median PFS/OS data and comparison to prior IRd studies. IRd is a well-tolerated and effective triplet regimen, often given as 2nd or 3rd-line therapy in RRMM, with results confirming the registration trial and other international RW-analyses.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144224102","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":"Three novel heterozygous ANK1 loss-of-function variants cause hereditary spherocytosis in Chinese families.","authors":"Yang Wang, Tao Liu, Wen Wang, Yuzhuopu Li, Li Xiao, Yan Xiang, Lan Huang, Yongjie Zhang, Jie Yu","doi":"10.1007/s00277-025-06408-9","DOIUrl":"https://doi.org/10.1007/s00277-025-06408-9","url":null,"abstract":"<p><strong>Background and purposes: </strong>Hereditary spherocytosis (HS) is an inherited hemolytic anemia caused by variants in red blood cell cytoskeleton proteins. Advances in sequencing technology have revealed numerous novel variants; however, accurate genome annotation remains a significant challenge. In precision medicine, the precise identification of variants responsible for genetic diseases is crucial. This study aims to clearly delineate pathogenic variants and provide targeted pathogenicity analyses.</p><p><strong>Methods: </strong>We analyzed clinical data from three unrelated Chinese families with HS. Our investigation delved into the pathogenicity and underlying mechanisms of these variants. In silico simulations and in vitro experiments were employed to assess the effects of the identified variants.</p><p><strong>Results: </strong>We identified three novel heterozygous variants in the ANK1 gene: c.558delG (p.R187Afs*66), c.728T > G (p.L243R), and c.3157 C > T (p.R1053X). In silico analysis indicated that these variants adversely affect the ankyrin-1. Functional studies demonstrated that these variants disrupt the synthesis of ankyrin-1, weakening its interaction with other red blood cell cytoskeleton proteins, which may lead to HS due to the loss of ankyrin-1 function.</p><p><strong>Conclusions: </strong>Our study offers valuable insights into the molecular mechanisms associated with HS linked to three de novo ANK1 variants. These findings deepen our understanding of the pathogenesis of HS and emphasize the critical role of genetic screening in the diagnosis of this condition.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144207507","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}