Annals of Hematology最新文献_第5页

First report of B-lymphoblastic leukemia harboring LRRFIP1::FGFR1 fusion: expanding the clinical spectrum of myeloid/lymphoid neoplasms with tyrosine kinase gene fusions. 首次报道含有LRRFIP1::FGFR1融合的b淋巴母细胞白血病：扩大了酪氨酸激酶基因融合的髓/淋巴肿瘤的临床谱。

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-30 DOI: 10.1007/s00277-025-06481-0

Xue Chen, Yuyue Ren, Yinglan Jin, Xiaoyun Li, Xiaoli Ma, Panxiang Cao, Yang Zhang, Fang Wang, Hongxing Liu, Wei Wang

引用次数: 0

Genotype-dependent albuminuria in adult sickle cell disease in Kinshasa. 金沙萨成人镰状细胞病的基因型依赖性蛋白尿

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-30 DOI: 10.1007/s00277-025-06362-6

Yannick Mompango Engole, Jean Robert Rissassi Makulo, Justine Busanga Bukabau, Yannick Mayamba Nlandu, Brady Makanzu, Yannick Mvita, Aliocha Nkodila, François Musungayi Kajingulu, Vieux Momeme Mokoli, Augustin Luzayadio Longo, Marie France Ingole Mboliasa, Clarisse Nsenga Nkondi, Daddy Mbiso Liombo, James Kalunga, Blaise Nkolomoni, Ange Ngonde, Ernest Kiswaya Sumaili

{"title":"Genotype-dependent albuminuria in adult sickle cell disease in Kinshasa.","authors":"Yannick Mompango Engole, Jean Robert Rissassi Makulo, Justine Busanga Bukabau, Yannick Mayamba Nlandu, Brady Makanzu, Yannick Mvita, Aliocha Nkodila, François Musungayi Kajingulu, Vieux Momeme Mokoli, Augustin Luzayadio Longo, Marie France Ingole Mboliasa, Clarisse Nsenga Nkondi, Daddy Mbiso Liombo, James Kalunga, Blaise Nkolomoni, Ange Ngonde, Ernest Kiswaya Sumaili","doi":"10.1007/s00277-025-06362-6","DOIUrl":"https://doi.org/10.1007/s00277-025-06362-6","url":null,"abstract":"Albuminuria, which depends on multiple factors, is common in patients with sickle cell disease and can progress to chronic kidney disease. In this study, we investigated the frequency and determinants of albuminuria according to sickle cell disease genotype. This multicentre cross-sectional analytical study of adults with stable sickle cell disease was conducted in Kinshasa. Genotypes were categorised as follows: homozygous, HbSS; heterozygous, HbAS; albuminuria, urinary albumin/creatinine ratio (mg/g): grade A1, < 30; grade A2:30-300; or grade A3, > 300. In total, 247 patients with sickle cell disease were included: 205 homozygous and 42 heterozygous. Albuminuria was prevalent in 50.5% of homozygous and 56.1% of heterozygous patients. The multivariate analysis revealed that the factors independently associated with albuminuria in the homozygous group were age ≥ 30 years (p = 0.037), leg ulcers (p = 0.010), hypertension (p = 0.038), and C-reactive protein level > 6 mg/L (p = 0.033). In the heterozygous group, only hypertension (p = 0.009), C-reactive protein > 6 mg/L (p = 0.006) and a history of vaso-occlusive crisis (p = 0.014) emerged as factors independent factors. More than half of patients with sickle cell disease had albuminuria, which was independently associated with hypertension and inflammation in both groups. Furthermore, there was also an association between in albuminuria and age ≥ 30 years, manifestations of vasculopathy in the homozygous group and a history of vaso-occlusive crisis in the heterozygous group.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144526142","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

Effectiveness of venetoclax based therapy in t(11;14) multiple myeloma with extensive extramedullary disease: a case report. venetoclax为基础治疗多发性骨髓瘤伴广泛髓外病变1例

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-27 DOI: 10.1007/s00277-025-06489-6

Kerstin Brinkert, Bernd Heinrich, Annamaria Brioli, Michael Heuser, Hans Heinrich Kreipe, Florian H Heidel, Arnold Ganser, Gernot Beutel

引用次数: 0

Avapritinib monotherapy induces rapid and deep remission of heavily treated, KIT D816H-mutated t(8;21) acute myeloid leukemia, a case report and literature review. 阿伐替尼单药治疗可诱导重度治疗的KIT d816h突变t（8;21）急性髓系白血病快速深度缓解1例并文献复习。

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-27 DOI: 10.1007/s00277-025-06388-w

Ted M Getz, Kamila Bakirhan, Stuart Seropian, Rory M Shallis

引用次数: 0

Daratumumab plus bortezomib and dexamethasone as a bridge to allogeneic transplantation in refractory T-cell lymphoblastic lymphoma. 达拉单抗联合硼替佐米和地塞米松作为难治性t细胞淋巴母细胞淋巴瘤同种异体移植的桥梁。

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-26 DOI: 10.1007/s00277-025-06474-z

Alessio Maria Edoardo Maraglino, Simona Sammassimo, Ginevra Lolli, Alice Clemente, Valentina Tabanelli, Rocco Pastano, Enrico Derenzini

引用次数: 0

Feasibility of allogeneic stem cell transplantation (HSCT) in patients with acute myeloid leukemia previously treated with CPX-351: report from a single center. 同种异体干细胞移植（HSCT）治疗急性髓系白血病患者的可行性：来自单一中心的报告

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-23 DOI: 10.1007/s00277-025-06462-3

Sabrina Giammarco, Patrizia Chiusolo, Elisabetta Metafuni, Maria Assunta Limongiello, Federica Sorà, Filippo Frioni, Luca Di Marino, Livio Pagano, Simona Sica

引用次数: 0

Optimizing hemophagocytic lymphohistiocytosis screening in children: validation of the HLH-Screen score. 优化儿童噬血细胞淋巴组织细胞增多症筛查：hhl - screen评分的验证。

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-20 DOI: 10.1007/s00277-025-06465-0

Xiao Li, Zhexuan Tang, Lifang Zhou, Xun Li, Lihui Deng, Haipeng Yan, Xiangyu Wang, Longlong Xie, Ting Luo, Yufan Yang, Xinping Zhang, Jiaotian Huang, Yefei Lei, Zhenghui Xiao, Xiulan Lu

{"title":"Optimizing hemophagocytic lymphohistiocytosis screening in children: validation of the HLH-Screen score.","authors":"Xiao Li, Zhexuan Tang, Lifang Zhou, Xun Li, Lihui Deng, Haipeng Yan, Xiangyu Wang, Longlong Xie, Ting Luo, Yufan Yang, Xinping Zhang, Jiaotian Huang, Yefei Lei, Zhenghui Xiao, Xiulan Lu","doi":"10.1007/s00277-025-06465-0","DOIUrl":"10.1007/s00277-025-06465-0","url":null,"abstract":"Purpose: A previously study proposed a three-step screening procedure for pediatric hemophagocytic lymphohistiocytosis (HLH). This procedure includes an HLH screening model referred to as HLH-screen. This study aims to validate the three-step screening procedure and the HLH-Screen in an independent cohort of children, as well as to optimize the screening process for HLH. Patients and methods. A multicenter retrospective study was conducted on children with fever or splenomegaly hospitalized in three hospitals in Hunan Province. The screening performance of the three-step screening procedure and the simplified procedure using HLH-Screen alone were evaluated. Clinical characteristics of patients misclassified as false negatives or false positives by HLH-Screen were analyzed. Results. Medical records of 5294 children with fever or splenomegaly were collected. The overall sensitivity and specificity of the three-step screening procedure were 91.0-93.7% and 91.5-91.7%, respectively. When directly applying the HLH-Screen to the study population, the sensitivity was 89.7-93.7%, and the specificity was 92.2-92.4%. Four false-negative cases lacked cytopenias, two lacked splenomegaly, and all four experienced less severe organ damage. The most common false positive diagnoses were malignancy complicated with sepsis (25.4%), infectious mononucleosis (23.9%), and malignancy complicated with respiratory infections (7.9%). Conclusion. Both screening procedures showed strong and consistent performance in screening for pediatric HLH. While the sensitivity of the three-step procedure slightly outperformed that of the HLH-Screen alone, using the HLH-Screen directly is more convenient. The HLH-Screen-based HLH screening procedure uses simple parameters to rapidly identify high-risk HLH patients, making it applicable in resource-limited settings.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144332367","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

Urinary metabolomics predicts early rituximab response in steroid-nonresponsive pediatric patients with primary immune thrombocytopenia. 尿代谢组学预测类固醇无反应的原发性免疫性血小板减少症儿童患者的早期利妥昔单抗反应。

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-20 DOI: 10.1007/s00277-025-06453-4

Yuncui Yu, Zhenping Chen, Jing Wei, Xingjuan Xie, Juntao Ouyang, Zheyan Lin, Jingyao Ma, Peng Guo, Runhui Wu, Jingang Gui

引用次数: 0

Clinical and prognostic significance of whole blood Epstein-Barr virus DNA in patients with primary hemophagocytic lymphohistiocytosis: a retrospective observational study from China. 全血eb病毒DNA检测在原发性噬血细胞淋巴组织细胞增多症患者中的临床和预后意义：一项来自中国的回顾性观察研究

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-19 DOI: 10.1007/s00277-025-06447-2

Liurui Dou, Jia Zhang, Lin Wu, Jingshi Wang, Zhao Wang

{"title":"Clinical and prognostic significance of whole blood Epstein-Barr virus DNA in patients with primary hemophagocytic lymphohistiocytosis: a retrospective observational study from China.","authors":"Liurui Dou, Jia Zhang, Lin Wu, Jingshi Wang, Zhao Wang","doi":"10.1007/s00277-025-06447-2","DOIUrl":"https://doi.org/10.1007/s00277-025-06447-2","url":null,"abstract":"Hemophagocytic lymphohistiocytosis (HLH) has been described as a threshold disease depending on triggering factors and the residual cytotoxic capacity of NK cells. This study aimed to investigate the clinical characteristics of Epstein-Barr virus (EBV)-triggered primary HLH and the prognostic value of EBV-DNA load in EBV-triggered primary HLH cases. We retrospectively analyzed the clinical data of 95 patients with primary HLH treated between January 2013 and January 2024. Based on the peripheral blood EBV status at initial diagnosis, 57 patients were categorized into the EBV-triggered primary HLH group and 38 patients into the non-EBV-triggered primary HLH group. Clinical and functional characteristics, response to treatment, and prognosis were compared between the two groups. Among patients with EBV-triggered primary HLH, the proportion of patients with familial HLH type 2 (FHL2) was significantly lower (P = 0.011), whereas the proportion of patients with X-linked lymphoproliferative disorder (XLP) was higher (P = 0.037). Functional assays showed that in primary HLH patients with gene defects in cytotoxic degranulation, the proportion of EBV-triggered primary HLH patients with reduced NK cell activity and degranulation function was significantly higher (P = 0.026 and P = 0.030). Importantly, multivariate Cox regression analysis identified EBV-DNA > 10,000 copies/mL as an independent risk factor affecting the prognosis of patients with EBV-triggered primary HLH, particularly in non-FHL cases (P = 0.045). EBV-triggered primary HLH is more prevalent in patients with XLP but less frequent in FHL2 patients. High EBV-DNA load is an adverse prognostic factor in EBV-triggered primary HLH patients.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324351","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

Safe administration of venetoclax-obinutuzumab in a chronic lymphocytic leukemia patient with Brugada syndrome. venetoclax-obinutuzumab在慢性淋巴细胞白血病合并Brugada综合征患者中的安全应用

IF 3 3区医学

Annals of Hematology Pub Date : 2025-06-19 DOI: 10.1007/s00277-025-06457-0

Valeria Cardinali, Stefano Coiro, Maria Paola Martelli, Paolo Sportoletti

{"title":"Safe administration of venetoclax-obinutuzumab in a chronic lymphocytic leukemia patient with Brugada syndrome.","authors":"Valeria Cardinali, Stefano Coiro, Maria Paola Martelli, Paolo Sportoletti","doi":"10.1007/s00277-025-06457-0","DOIUrl":"https://doi.org/10.1007/s00277-025-06457-0","url":null,"abstract":"Brugada syndrome (BS) is a genetic condition that predisposes individuals to life-threatening arrhythmias, posing a challenge in the management of chronic lymphocytic leukemia (CLL). While BTK inhibitors have been associated with ventricular arrhythmias, data on the cardiac safety of venetoclax and obinutuzumab in BS patients remain limited. We describe the case of a 60-year-old CLL patient with a baseline type 2 Brugada ECG pattern who was treated with venetoclax plus obinutuzumab due to concerns over BTK inhibitor-associated cardiac risks. During the first obinutuzumab infusion, the patient experienced an infusion-related reaction with hypotension, hypokalemia, and transient conversion to a type 1 Brugada ECG pattern. Supportive measures, including electrolyte correction, led to ECG normalization. Venetoclax was well tolerated, and the patient achieved a complete response with undetectable measurable residual disease. This case underscores the importance of cardiac monitoring in CLL patients with BS and suggests that venetoclax plus obinutuzumab may represent a safe and effective therapeutic alternative when BTK inhibitors are contraindicated.","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324355","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