Annals of Hematology最新文献

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Impact of chromosomal aberrations detected by chromosome banding analysis in symptomatic Waldenström's macroglobulinemia. 在有症状的瓦尔登斯特伦巨球蛋白血症患者中通过染色体带分析检测到的染色体畸变的影响。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-16 DOI: 10.1007/s00277-024-06041-y
Kenichi Ito, Tomoko Kitagawa, Kunihiko Harada, Kazuhiko Hirano, Naohiro Sekiguchi
{"title":"Impact of chromosomal aberrations detected by chromosome banding analysis in symptomatic Waldenström's macroglobulinemia.","authors":"Kenichi Ito, Tomoko Kitagawa, Kunihiko Harada, Kazuhiko Hirano, Naohiro Sekiguchi","doi":"10.1007/s00277-024-06041-y","DOIUrl":"https://doi.org/10.1007/s00277-024-06041-y","url":null,"abstract":"<p><p>The clinicopathologic features and prognostic impact of MYD88 L265P (MYD<sup>L265P</sup>) and CXCR4 mutations (CXCR4<sup>Mut</sup>) have been well reported, although little is known regarding the impact of chromosomal aberrations (CA) detected by chromosome banding analysis (CBA) in symptomatic Waldenström's macroglobulinemia (sWM). Thus, we investigated the clinicopathologic features and prognostic impact in sWM with CAs identified by CBA. We retrospectively analyzed the clinicopathologic results and genomic alterations by droplet digital PCR, fluorescence in situ hybridization (FISH), and CBA using the G-banding method in bone marrow samples from sWM patients collected between April 2010 and March 2024 at our institute. The relationship between CAs and clinicopathologic features was evaluated, as well as the time to next treatment (TTNT). Thirty-five patients were enrolled. The median age was 71 years, and the median hemoglobin level was 10.1 g/dL. The median serum IgM and M-protein levels were 3,009 mg/dL and 2.95 g/dL, respectively. MYD<sup>L265P</sup> was found in 30/35 patients (85.7%), whereas CXCR4<sup>Mut</sup> was found in 3/35 patients (8.6%). Deletion 6q identified by FISH in 5/18 patients (28%), and CAs using CBA in 9/34 patients (26%), including 4/34 (12%) complex karyotypes. sWM with CAs had more anemia (p = 0.04) and hypoalbuminemia (p = 0.007), in addition to higher serum M-protein and IgM levels (p = 0.03). With a median follow-up of 73 months, the median TTNT in patients with and without CAs was 27 and 68 months, respectively. CAs with CBA may be associated with clinical aggressiveness and shorter TTNT in sWM.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456648","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
Clinical data and MRI features-based nomogram for differentiation of central nervous system infection and central nervous system involvement in hematological malignancy. 基于临床数据和磁共振成像特征的血液恶性肿瘤中枢神经系统感染和中枢神经系统受累鉴别提名图。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-16 DOI: 10.1007/s00277-024-06036-9
Huiming Yi, Yansong Ren, Shuping Zhang, Chunhui Xu, Wenyu Yang, Xin Chen, Xiaoxue Wang, Ying Zhong, Yingchang Mi, Sizhou Feng
{"title":"Clinical data and MRI features-based nomogram for differentiation of central nervous system infection and central nervous system involvement in hematological malignancy.","authors":"Huiming Yi, Yansong Ren, Shuping Zhang, Chunhui Xu, Wenyu Yang, Xin Chen, Xiaoxue Wang, Ying Zhong, Yingchang Mi, Sizhou Feng","doi":"10.1007/s00277-024-06036-9","DOIUrl":"https://doi.org/10.1007/s00277-024-06036-9","url":null,"abstract":"<p><p>Central nervous system leukemia (CNSL) and central nervous system infection (CNSI) are the most important complications in patients with acute leukemia (AL). However, the differential diagnosis could represent a major challenge since the two disorders are all heterogeneous entities with overlapping clinical characteristics and radiological appearances. In this paper, we conduct a retrospective study to develop a model based on clinical data and magnetic resonance imaging (MRI) to distinguish CNSL from CNSI. A total of 108 patients with AL who underwent cranial MRI between January 2020 and December 2023 in our hospital were included. Univariate and multivariate logistic regression analyses were used to determine the independent predictors. A nomogram was developed based on the predictors, and the performance of the nomogram was evaluated by the area under the receiver operating characteristic (ROC) curve. The validation cohort was used to test the predictive model. Hyperleukocytosis at initial diagnosis, marrow state, fever, conscious disturbance, coinfection in other sites and MRI (parenchyma type) were identified as independent factors. A nomogram was constructed and the discrimination was presented as AUC = 0.947 (95% CI 0.9105-0.984). Calibration of the nomogram showed that the predicted probability matched the actual probability well.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456646","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
Ischemic stroke as a presenting feature of promyelocytic blast phase in chronic myeloid leukemia - an uncommon presentation: a case report and literature review in the post imatinib era. 缺血性中风作为慢性粒细胞白血病早幼粒细胞暴发性期的一种表现特征--一种不常见的表现:后伊马替尼时代的病例报告和文献综述。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-16 DOI: 10.1007/s00277-024-06044-9
Swapnil Tripathi, Vinu Balraam K V, Amiya Ranjan Nayak, Richa Chauhan, Pradeep Kumar, Jasmita Dass, Priyanka Naranje, Mukul Aggarwal
{"title":"Ischemic stroke as a presenting feature of promyelocytic blast phase in chronic myeloid leukemia - an uncommon presentation: a case report and literature review in the post imatinib era.","authors":"Swapnil Tripathi, Vinu Balraam K V, Amiya Ranjan Nayak, Richa Chauhan, Pradeep Kumar, Jasmita Dass, Priyanka Naranje, Mukul Aggarwal","doi":"10.1007/s00277-024-06044-9","DOIUrl":"https://doi.org/10.1007/s00277-024-06044-9","url":null,"abstract":"<p><p>Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm (MPN characterized by reciprocal translocation in the ABL1 and BCR region of chromosomes 9 and 22 respectively. Progression to the blast phase in chronic myeloid leukemia results in a poorer prognosis. It can be of either myeloid, lymphoid or a mixed lineage. Progression to the promyelocytic blast phase is very rare, and there are no evidence-based guidelines for its management. Thrombosis in CML is not well defined. Thrombosis can be seen in patients with acute promyelocytic leukemia (APL) with venous thrombosis (VTE) being more common than arterial thrombosis. Ischemic stroke as the presenting feature of blast phase progression in CML is extremely rare. We report a case of CML who presented to us with acute ischemic stroke and subsequently was diagnosed as CML transformed to the promyelocytic blast phase. She was successfully treated with dasatinib along with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO).</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456649","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 comprehensive analysis of the major specificities of anti-human T lymphocyte porcine immunoglobulin (p-ATG). 抗人 T 淋巴细胞猪免疫球蛋白(p-ATG)主要特异性的综合分析。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-15 DOI: 10.1007/s00277-024-06028-9
Haoyong Zou, Wenqu Yin, Peng Geng, Li Lin, Xilin Nie, Zui Tao, Gang Chen, Bin Chen, Hao Feng, Kuanhong Xu, Zhi Zhang
{"title":"A comprehensive analysis of the major specificities of anti-human T lymphocyte porcine immunoglobulin (p-ATG).","authors":"Haoyong Zou, Wenqu Yin, Peng Geng, Li Lin, Xilin Nie, Zui Tao, Gang Chen, Bin Chen, Hao Feng, Kuanhong Xu, Zhi Zhang","doi":"10.1007/s00277-024-06028-9","DOIUrl":"https://doi.org/10.1007/s00277-024-06028-9","url":null,"abstract":"<p><strong>Background: </strong>Anti-human T lymphocyte porcine immunoglobulin (p-ATG) is a potent immunosuppressive agent derived from porcine sources used in various immunotherapy applications. It is compared with similar products derived from other species, such as rabbit anti-thymocyte globulin (r-ATG) and ATG-Fresenius (ATG-F), which have distinct biological and therapeutic properties. This study aims to elucidate the mechanisms of action and comparative efficacy of p-ATG in relation to r-ATG and ATG-F through a comprehensive in vitro analysis.</p><p><strong>Methods: </strong>A comparative analysis of p-ATG, r-ATG and ATG-F was performed, focusing on E rosette inhibitory potency, lymphocyte toxic potency, blocking activities of 24 CD molecules, and flow quantitative potency. Flow cytometric analysis was used to quantify these characteristics and assess the potency of the immunoglobulins.</p><p><strong>Results: </strong>p-ATG exhibited lower E rosette inhibitory and lymphocyte toxic potencies compared to r-ATG but was significantly more potent than ATG-F at equivalent concentrations. At protein concentrations above 12.5 µg/mL, p-ATG showed slightly lower potency than r-ATG and much higher potency than ATG-F in flow cytometry assays. Both p-ATG and r-ATG exhibited similar killing effects on lymphocytes within the peripheral blood mononuclear cells (PBMCs), including CD3 + T cells, with a dose-dependent response. Notably, p-ATG displayed more pronounced blocking activities against CD8, CD99, and TCR α/β compared to r-ATG.</p><p><strong>Conclusion: </strong>p-ATG offers certain advantages over r-ATG and ATG-F, particularly in its ability to inhibit specific CD molecules and its overall potency in immunosuppressive assays, providing valuable insights for assisting clinical decision-making regarding the selection of ATG types based on patient-specific needs and treatment objectives.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456643","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
Different prognosis according to treatment in patients with acute promyelocytic leukemia: How the outcome changed over time. 急性早幼粒细胞白血病患者的预后因治疗方法而异:随着时间的推移,结果如何变化。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-15 DOI: 10.1007/s00277-024-06014-1
Emilia Scalzulli, Alessandro Costa, Ida Carmosino, Paolo Musiu, Maria Laura Bisegna, Maria Stefania De Propris, Claudia Ielo, Daniela Diverio, Clara Minotti, Saveria Capria, Roberto Latagliata, Maurizio Martelli, Massimo Breccia
{"title":"Different prognosis according to treatment in patients with acute promyelocytic leukemia: How the outcome changed over time.","authors":"Emilia Scalzulli, Alessandro Costa, Ida Carmosino, Paolo Musiu, Maria Laura Bisegna, Maria Stefania De Propris, Claudia Ielo, Daniela Diverio, Clara Minotti, Saveria Capria, Roberto Latagliata, Maurizio Martelli, Massimo Breccia","doi":"10.1007/s00277-024-06014-1","DOIUrl":"https://doi.org/10.1007/s00277-024-06014-1","url":null,"abstract":"<p><p>A comprehensive analysis of 220 patients diagnosed with APL between 1993 and 2022 is here reported. Overall, 214 patients (97.2%) received induction therapy. Complete response (CR) was achieved in 97.4%, 100%, 100%, and 27% of patients treated with AIDA protocol, AIDA + Ara-C, ATRA + ATO, and ATRA monotherapy, respectively. Molecular complete response (CR<sub>MRD</sub>-) was achieved in 96.8% cases, and 142 patients proceeded to maintenance therapy. Overall, the 3-year and 5-year overall survival (OS) rates were 80.8% (95% CI, 78.1-83.5) and 79.1% (95% CI, 76.4-81.8), respectively. Considering only patients who completed induction and maintenance therapy, the 5-year OS rates were 82.1% (95% CI, 77.5-86.7) for the AIDA0493 cohort, 87.5% (95% CI, 84.4-91.1) for the AIDA2000 cohort, and 100% for the APL0406 cohort (p = 0.044). Additionally, the disease-free survival (DFS) rates were 65.7% (95% CI, 60.4-70.9), 70% (95% CI, 65.8-75.2), and 95.1% (95% CI, 91.7-98.5) (p = 0.016), respectively. Among low and intermediate-risk patients, age > 70 years (p = 0.027) and relapse (p < 0.001) were significantly associated with reduced outcomes. This study contributes to the advancement of our understanding of APL treatment, underscoring the ongoing need for research to enhance outcomes and explore new therapeutic approaches and prognostic factors.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456647","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
Peculiar hyper vascular manifestations in idiopathic multicentric castleman disease without tafro syndrome: a case report. 无塔夫罗综合征的特发性多中心卡斯特曼病的特殊高血管表现:病例报告。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-14 DOI: 10.1007/s00277-024-06040-z
Rahmeen Khisal Jilani, Muhammad Rizwan Saleem, Shumaila Akhtar, Wajeha Jawad
{"title":"Peculiar hyper vascular manifestations in idiopathic multicentric castleman disease without tafro syndrome: a case report.","authors":"Rahmeen Khisal Jilani, Muhammad Rizwan Saleem, Shumaila Akhtar, Wajeha Jawad","doi":"10.1007/s00277-024-06040-z","DOIUrl":"https://doi.org/10.1007/s00277-024-06040-z","url":null,"abstract":"<p><strong>Background: </strong>Castleman disease affects lymph nodes with abnormal cell growth. It has unicentric (single node) Castleman disease (UCD) and multicentric (multiple nodes) Castleman disease (MCD) forms. MCD is systemic, with diverse symptoms, necessitating systemic treatment. Idiopathic MCD (iMCD) clinical subtypes are divided into iMCD- not otherwise specified (NOS) and iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticular fibrosis, organomegaly). UCD, iMCD-NOS, and iMCD-TAFRO mainly exhibit histopathology of hyaline vascular type, plasma cell type, and hyper vascular type, respectively.</p><p><strong>Case presentation: </strong>A 21-year-old female with no comorbidities presented to the outpatient department (OPD) with left inguinal swelling, gradually growing over four years, accompanied by fever and weight loss. Her past medical history included pulmonary TB 5 years prior and miscarriages. Vitals are within normal limits. Examination revealed a tender, nonreducible inguinal lump and a smaller neck swelling. Serological tests for infections were negative. Imaging revealed enlarged lymph nodes. Biopsy confirmed Castleman disease of the hyper vascular type. We performed surgical removal of the enlarged lymph nodes followed by close regular follow-up along with potential chemotherapy for relapse.</p><p><strong>Conclusion: </strong>Hyper vascular type of the lymph node histology in Idiopathic multicentric Castleman disease without TAFRO syndrome must be considered a differential diagnosis in lymphoproliferative disease.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456653","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
Olverembatinib treatment in adult patients with newly diagnosed Philadelphia chromosome-positive acute lymphoblastic leukemia 新诊断的费城染色体阳性急性淋巴细胞白血病成人患者的奥韦拉替尼治疗。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-14 DOI: 10.1007/s00277-024-06027-w
Yiyan Zhu, Jiayi Huang, Ying Wang, Yue Han, Shengli Xue, Yonggong Yang, Yu Zhu, Wenzhi Cai, Suning Chen
{"title":"Olverembatinib treatment in adult patients with newly diagnosed Philadelphia chromosome-positive acute lymphoblastic leukemia","authors":"Yiyan Zhu,&nbsp;Jiayi Huang,&nbsp;Ying Wang,&nbsp;Yue Han,&nbsp;Shengli Xue,&nbsp;Yonggong Yang,&nbsp;Yu Zhu,&nbsp;Wenzhi Cai,&nbsp;Suning Chen","doi":"10.1007/s00277-024-06027-w","DOIUrl":"10.1007/s00277-024-06027-w","url":null,"abstract":"<p>Olverembatinib is a novel orally administered third-generation tyrosine kinase inhibitor (TKI) with definitive responses in T315I-mutant chronic myeloid leukemia (CML) patients. However, its value in newly diagnosed Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph + ALL) remained unclarified. In this multiple-center study, 20 patients with <i>de novo</i> Ph + ALL were treated with olverembatinib-based regimens as frontline therapy. All patients acquired complete remission (CR) after induction. 85% of patients achieved complete molecular response (CMR) within three months, contributed mainly by the addition of blinatumomab. A total of 45% of patients experienced mild hematological treatment-related adverse events (TRAEs). Olverembatinib-based treatment led to promising outcomes in <i>de novo</i> Ph + ALL patients but warranted further studies to investigate the best-combined strategy.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"103 11","pages":"4643 - 4648"},"PeriodicalIF":3.0,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142456652","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
Manifestations and outcomes of digestive tract involvement in adult Langerhans cell histiocytosis 成人朗格汉斯细胞组织细胞增生症消化道受累的表现和结果。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-09 DOI: 10.1007/s00277-024-06034-x
Qing Shang, Long Chang, Min Lang, Zheng-zheng Liu, He Lin, Jin-hua Zhao, Yue Li, Xin-xin Cao
{"title":"Manifestations and outcomes of digestive tract involvement in adult Langerhans cell histiocytosis","authors":"Qing Shang,&nbsp;Long Chang,&nbsp;Min Lang,&nbsp;Zheng-zheng Liu,&nbsp;He Lin,&nbsp;Jin-hua Zhao,&nbsp;Yue Li,&nbsp;Xin-xin Cao","doi":"10.1007/s00277-024-06034-x","DOIUrl":"10.1007/s00277-024-06034-x","url":null,"abstract":"<div><p>Langerhans cell histiocytosis (LCH) is a heterogeneous histiocytosis characterized by proliferation of Langerhans cells. While less common, manifestations of digestive tract involvement in LCH remain largely unrevealed. We conducted a retrospective analysis of demographics, clinical, endoscopic, genetic and follow-up data from 13 adult patients with pathologically confirmed gastrointestinal involvement of LCH (LCH-GI), in a single-center cohort of 465 patients. Digestive tract involvement was observed in 2.80% of LCH patients. At LCH-GI diagnosis, 7 patients (53.8%) had unifocal lesions, and 6 patients (46.2%) had multisystem disease. 6 patients (46.2%) experienced no gastrointestinal symptoms at LCH-GI onset, while others were symptomatic. Stomach was most commonly affected (61.5%), followed by esophagus (23.1%), colon (7.7%) and anus (7.7%). Endoscopic findings varied among 12 patients, including submucosal bulge (8 patients, 66.7%) and non-bulging lesions (4 patients, 33.3%) such as erosions, coarse granular mucosa, and regional abnormal coloration. Among 8 patients with genetic analysis, <i>BRAF</i><sup><i>V600E</i></sup> mutation was detected in 5 patients (62.5%). The estimated 1-year overall survival rate was 91.7%. Progression-free survival of patients with submucosal bulges under endoscopy was significantly better than those with non-bulging lesions. This study presents 13 cases of LCH with digestive tract involvement. We emphasize the importance of endoscopy and biopsy for pathological examination of lesions such as submucosal bulges and erosions under endoscopy to assist in early detection of LCH. Comprehensive systemic assessment and regular endoscopic monitoring are essential in patient management. Treatment should be individualized with dynamic adjustments during follow-up.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"103 11","pages":"4459 - 4466"},"PeriodicalIF":3.0,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142387464","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
Distinct clinical profiles and patient outcomes in aCML and CNL. aCML 和 CNL 不同的临床特征和患者预后。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-08 DOI: 10.1007/s00277-024-06032-z
Yingxin Sun, Qinrong Wang, Zhiyu Zhang, Qian Wang, Jiannong Cen, Mingqing Zhu, Jinlan Pan, Dandan Liu, Hongjie Shen, Yifeng Cai, Suning Chen
{"title":"Distinct clinical profiles and patient outcomes in aCML and CNL.","authors":"Yingxin Sun, Qinrong Wang, Zhiyu Zhang, Qian Wang, Jiannong Cen, Mingqing Zhu, Jinlan Pan, Dandan Liu, Hongjie Shen, Yifeng Cai, Suning Chen","doi":"10.1007/s00277-024-06032-z","DOIUrl":"https://doi.org/10.1007/s00277-024-06032-z","url":null,"abstract":"<p><p>The classification of atypical chronic myeloid leukemia (aCML) and chronic neutrophilic leukemia (CNL) as a single disease entity remains a topic of debate. To elucidate the characteristics of both entities, this retrospective cohort study was conducted, encompassing 36 cases of aCML and 18 cases of CNL. We discovered that aCML and CNL presented distinct blood counts, genetics, molecular profiles and outcomes. Specifically, hemoglobin levels (P < 0.001) and platelet counts (P < 0.001) were significantly lower in aCML cases than in CNL cases, with no significant difference in mean white blood cells (P = 0.637). The proportion of abnormal karyotypes was higher in aCML cases compared with CNL cases (P = 0.010). Notably, we found that aCML and CNL showed distinct gene expression profiles by transcriptome sequencing technology. The median follow-up duration for the entire cohort was 8 months (rang 0.4 to 36.6 months), and the median overall survival (OS) was significantly shorter in aCML cases (7.3 months, 95%CI 5.4 to 20.5 months) than in CNL cases (median OS not reached). The one-year OS rate for aCML patients was 31.0% (9/29), compared to 92.9% (13/14) for CNL patients. In conclusion, our study supports the notion that aCML and CNL are indeed distinct disease entities characterized by unique hematological features and clinical outcomes.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142387463","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
Secondary prophylaxis of venous thromboembolism (VTE) with low dose apixaban or rivaroxaban in major-thrombophilia carriers 使用小剂量阿哌沙班或利伐沙班对主要血栓性疾病携带者进行静脉血栓栓塞症(VTE)二级预防。
IF 3 3区 医学
Annals of Hematology Pub Date : 2024-10-08 DOI: 10.1007/s00277-024-06021-2
Alessandro Laganà, Silvia Sorella, Ludovica Fucci, Cristina Santoro, Silvio Ligia, Rosaria Mormile, Erminia Baldacci, Antonio Chistolini
{"title":"Secondary prophylaxis of venous thromboembolism (VTE) with low dose apixaban or rivaroxaban in major-thrombophilia carriers","authors":"Alessandro Laganà,&nbsp;Silvia Sorella,&nbsp;Ludovica Fucci,&nbsp;Cristina Santoro,&nbsp;Silvio Ligia,&nbsp;Rosaria Mormile,&nbsp;Erminia Baldacci,&nbsp;Antonio Chistolini","doi":"10.1007/s00277-024-06021-2","DOIUrl":"10.1007/s00277-024-06021-2","url":null,"abstract":"<div><p>Direct oral anticoagulants (DOACs) are widely used for treatment and secondary prophylaxis of venous thromboembolism (VTE) and represent the gold standard for VTE secondary prophylaxis, with low-intensity DOACs administration becoming increasingly used worldwide in such scenario. Albeit widespread DOACs usage there are few literature data regarding their efficacy and safety in major thrombophilia carriers and almost no data is available for low intensity apixaban and rivaroxaban as secondary VTE prophylaxis in such patients. The aim of our study is to evaluate and confront the efficacy and safety of low-dose DOACs for VTE secondary prophylaxis, in major thrombophilia carriers vs patients at high risk of VTE recurrence for other reasons. We retrospectively evaluated patients who required long-term anticoagulant secondary prophylaxis to prevent recurrent VTE, treated with apixaban 2.5 mg BID or rivaroxaban 10 mg daily and that were screened for thrombophilia. The examined patients were 339. Baseline characteristics such as sex, age, obesity rate, smoking, number of previous VTEs and comorbidities (such as cardiovascular diseases, diabetes, mild CKD) were equally distributed in either group. The median low-dose DOACs administration time was 19.20 months (IQR 12.17–35.67). During low-dose DOACs treatment, 13 (3.8%) VTE recurrences were observed; 14 bleeding events were registered (4,1%), with no major bleeding (MB), 6 clinically relevant non major bleeding (CRNMB) (1.8%) and 8 minor bleeding (2.3%). No statistically significant difference in the rate of VTE recurrence and/or bleeding events emerged between major thrombophilia carriers and non-major thrombophilia carriers. In multivariate analysis increased risk of VTE recurrence was found for patients with cardiovascular comorbidities (HR 4.00 – <i>p</i> = 0.034) and for patients with more than one previous VTE episode (HR 5.14 — <i>p</i> = 0.034). Our data suggest that low-dose DOACs may be effective and safe in secondary VTE prophylaxis for carriers of major thrombophilia with no increase in VTE recurrence and/or bleeding risk.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"103 11","pages":"4731 - 4739"},"PeriodicalIF":3.0,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142387465","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}
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