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Data-driven, harmonised classification system for myelodysplastic syndromes: a consensus paper from the International Consortium for Myelodysplastic Syndromes. 数据驱动的骨髓增生异常综合征统一分类系统:骨髓增生异常综合征国际联盟共识文件。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-10-09 DOI: 10.1016/S2352-3026(24)00251-5
Rami S Komrokji, Luca Lanino, Somedeb Ball, Jan P Bewersdorf, Monia Marchetti, Giulia Maggioni, Erica Travaglino, Najla H Al Ali, Pierre Fenaux, Uwe Platzbecker, Valeria Santini, Maria Diez-Campelo, Avani Singh, Akriti G Jain, Luis E Aguirre, Sarah M Tinsley-Vance, Zaker I Schwabkey, Onyee Chan, Zhouer Xie, Andrew M Brunner, Andrew T Kuykendall, John M Bennett, Rena Buckstein, Rafael Bejar, Hetty E Carraway, Amy E DeZern, Elizabeth A Griffiths, Stephanie Halene, Robert P Hasserjian, Jeffrey Lancet, Alan F List, Sanam Loghavi, Olatoyosi Odenike, Eric Padron, Mrinal M Patnaik, Gail J Roboz, Maximilian Stahl, Mikkael A Sekeres, David P Steensma, Michael R Savona, Justin Taylor, Mina L Xu, Kendra Sweet, David A Sallman, Stephen D Nimer, Christopher S Hourigan, Andrew H Wei, Elisabetta Sauta, Saverio D'Amico, Gianluca Asti, Gastone Castellani, Mattia Delleani, Alessia Campagna, Uma M Borate, Guillermo Sanz, Fabio Efficace, Steven D Gore, Tae Kon Kim, Navel Daver, Guillermo Garcia-Manero, Maria Rozman, Alberto Orfao, Sa A Wang, M Kathryn Foucar, Ulrich Germing, Torsten Haferlach, Phillip Scheinberg, Yasushi Miyazaki, Marcelo Iastrebner, Austin Kulasekararaj, Thomas Cluzeau, Shahram Kordasti, Arjan A van de Loosdrecht, Lionel Ades, Amer M Zeidan, Matteo G Della Porta

The WHO and International Consensus Classification 2022 classifications of myelodysplastic syndromes enhance diagnostic precision and refine decision-making processes in these diseases. However, some discrepancies still exist and potentially cause inconsistency in their adoption in a clinical setting. We adopted a data-driven approach to provide a harmonisation between these two classification systems. We investigated the importance of genomic features and their effect on the cluster assignment process to define harmonised entity labels. A panel of expert haematologists, haematopathologists, and data scientists who are members of the International Consortium for Myelodysplastic Syndromes was formed and a modified Delphi consensus process was adopted to harmonise morphologically defined categories without a distinct genomic profile. The panel held regular online meetings and participated in a two-round survey using an online voting tool. We identified nine clusters with distinct genomic features. The cluster of highest hierarchical importance was characterised by biallelic TP53 inactivation. Cluster assignment was irrespective of blast count. Individuals with monoallelic TP53 inactivation were assigned to other clusters. Hierarchically, the second most important group included myelodysplastic syndromes with del(5q). Isolated del(5q) and less than 5% of blast cells in the bone marrow were the most relevant label-defining features. The third most important cluster included myelodysplastic syndromes with mutated SF3B1. The absence of isolated del(5q), del(7q)/-7, abn3q26.2, complex karyotype, RUNX1 mutations, or biallelic TP53 were the basis for a harmonised label of this category. Morphologically defined myelodysplastic syndrome entities showed large genomic heterogeneity that was not efficiently captured by single-lineage versus multilineage dysplasia, marrow blasts, hypocellularity, or fibrosis. We investigated the biological continuum between myelodysplastic syndromes with more than 10% bone marrow blasts and acute myeloid leukaemia, and found only a partial overlap in genetic features. After the survey, myelodysplastic syndromes with low blasts (ie, less than 5%) and myelodysplastic syndromes with increased blasts (ie, 5% or more) were recognised as disease entities. Our data-driven approach can efficiently harmonise current classifications of myelodysplastic syndromes and provide a reference for patient management in a real-world setting.

世界卫生组织和国际共识分类 2022 对骨髓增生异常综合征的分类提高了诊断的精确性,并完善了这些疾病的决策过程。然而,它们之间仍存在一些差异,可能导致在临床应用中的不一致性。我们采用了一种数据驱动的方法来协调这两种分类系统。我们研究了基因组特征的重要性及其对聚类分配过程的影响,以定义统一的实体标签。我们成立了一个由骨髓增生异常综合征国际联盟成员中的血液学专家、血液病理学专家和数据科学家组成的专家小组,并采用修改后的德尔菲共识流程来协调形态学上定义的、没有明显基因组特征的类别。专家小组定期举行在线会议,并使用在线投票工具参与了两轮调查。我们确定了九个具有明显基因组特征的群组。分层重要性最高的簇群以双拷贝 TP53 失活为特征。聚类分配与突变计数无关。单复制 TP53 失活的个体被分配到其他群组。从层次上看,第二大类包括骨髓增生异常综合征伴del(5q)。孤立的 del(5q)和骨髓中少于 5%的暴发性细胞是最相关的标签定义特征。第三大类包括SF3B1突变的骨髓增生异常综合征,不存在孤立的del(5q)、del(7q)/-7、abn3q26.2、复杂核型、RUNX1突变或双倍TP53是该类疾病统一标签的基础。从形态学角度定义的骨髓增生异常综合征实体显示出巨大的基因组异质性,而单系与多系发育不良、骨髓囊胚、细胞减少或纤维化并不能有效地反映这种异质性。我们研究了骨髓增生异常综合征(骨髓泡超过 10%)与急性髓性白血病之间的生物学连续性,发现两者的遗传特征只有部分重叠。经过调查,低血小板(即低于 5%)骨髓增生异常综合征和血小板增多(即 5%或以上)骨髓增生异常综合征被认定为疾病实体。我们的数据驱动方法可有效协调骨髓增生异常综合征的现有分类,并为现实世界中的患者管理提供参考。
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引用次数: 0
Renal colocalisation of Rosai-Dorfman-Destombes disease and secondary AA amyloidosis successfully treated with lenalidomide and dexamethasone. 来那度胺和地塞米松成功治疗罗赛-多夫曼-德斯坦贝病和继发性AA淀粉样变性的肾脏共同病变。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-01 DOI: 10.1016/S2352-3026(24)00271-0
Rayane Benyahia, Charlotte Syrykh, Clotilde Gaible, Julie Belliere, Magali Colombat, Audrey Delas, Jérémie Dion, Pierre Cougoul
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引用次数: 0
Maternal and obstetric outcomes in women with pregnancy-associated haematological malignancies: an observational nationwide cohort study. 妊娠相关血液恶性肿瘤妇女的产妇和产科结局:一项全国范围的观察性队列研究。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-10-07 DOI: 10.1016/S2352-3026(24)00288-6
Pierre Pinson, Ismael Boussaid, Justine Decroocq, Laurent Chouchana, Gary Birsen, Mathilde Barrois, Vassilis Tsatsaris, Charlotte Godeberge, Jeremie Zerbit, Barbara Burroni, Frederic Pene, Laurence Huynh, Caroline Charlier, Jerome Tamburini, Nathanael Beeker, Mathis Collier, Didier Bouscary, Jean Marc Treluyer, Rudy Birsen
<p><strong>Background: </strong>Pregnancy-associated haematological malignancy is a rare event; therefore, data available to guide the treatment are scarce. We aimed to evaluate the incidence, overall survival, and maternal morbidity and mortality of women with pregnancy-associated haematological malignancies.</p><p><strong>Methods: </strong>We conducted a nationwide observational cohort study using the French National Healthcare Data System (SNDS), a health-care administrative database covering up to 99% of the French population. We included all pregnancies in France ending between Jan 1, 2012, and Dec 31, 2022. Pregnancies with terminations or miscarriages managed on an outpatient basis, and women with a history of haematological malignancies before pregnancy were excluded. A Cox proportional hazards model was used to assess overall survival, defined as the date of haematological malignancy diagnosis to either death or the end of the study follow-up, in the haematological malignancy during pregnancy group (pregnancies with a diagnosis of haematological malignancy during pregnancy) compared with the haematological malignancy post-pregnancy group (pregnancies with a diagnosis of haematological malignancy in the year following pregnancy). Severe maternal morbidity was compared in the haematological malignancy during pregnancy group versus the reference group (pregnancies without a history of haematological malignancy or a diagnosis of pregnancy-associated haematological malignancy). Births were classified as very preterm (<32 weeks of pregnancy), preterm (32-36 weeks), and term (≥37 weeks) and compared in the haematological malignancy during pregnancy group versus the reference group. Inverse probability weighting (IPW) was used for confounder adjustment, using maternal age (categorised), comorbidities, socioeconomic status, and year of delivery (as a category).</p><p><strong>Findings: </strong>Of 9 996 523 pregnancies in 5 995 235 women, 1366 pregnancy-associated haematological malignancies were identified: 413 during pregnancy (4·13 per 100 000 pregnancies) and 953 (9·53 per 100 000 pregnancies) within 12 months of the end of pregnancy (post-pregnancy). No significant differences in overall survival were observed between the haematological malignancy during and post-pregnancy groups across all types of haematological malignancy (IPW-adjusted hazard ratio 0·91 [95% CI 0·62-1·34], p=0·63), specifically for Hodgkin lymphoma (0·56 [0·07-4·53], p=0·59), aggressive B-cell non-Hodgkin lymphoma (0·52 [0·12-2·38], p=0·40), and acute leukaemia alone (0·84 [0·50-1·41], p=0·51). Severe maternal morbidity was more frequent in the haematological malignancy during pregnancy group than in the reference group (86 [26·2%] of 328 completed pregnancies vs 120 335 [1·5%] of 7 945 909 completed pregnancies; IPW-adjusted odds ratio 22·71 [95% CI 17·72-29·10], p<0·0001). We observed an increase in very preterm birth (32 [9·8%] vs 92 712 [1·2%]; IPW-adjusted odds ratio
背景:妊娠相关性血液恶性肿瘤是一种罕见的疾病,因此可用于指导治疗的数据很少。我们旨在评估妊娠相关血液恶性肿瘤妇女的发病率、总生存率、孕产妇发病率和死亡率:我们利用法国国家医疗保健数据系统(SNDS)开展了一项全国性的观察性队列研究,该系统是一个医疗保健管理数据库,覆盖了法国 99% 的人口。我们纳入了法国2012年1月1日至2022年12月31日期间的所有妊娠。不包括门诊终止妊娠或流产的孕妇,也不包括怀孕前有血液恶性肿瘤病史的妇女。采用考克斯比例危险模型评估妊娠期血液恶性肿瘤组(妊娠期诊断为血液恶性肿瘤的孕妇)与妊娠后血液恶性肿瘤组(妊娠后一年诊断为血液恶性肿瘤的孕妇)的总生存率,总生存率的定义为从血液恶性肿瘤诊断日期到死亡或研究随访结束的时间。妊娠期血液恶性肿瘤组与参照组(无血液恶性肿瘤病史或诊断出与妊娠相关的血液恶性肿瘤的孕妇)的孕产妇严重发病率进行了比较。早产儿被归类为极早产儿(研究结果显示:在 9 996 523 名孕妇中,早产儿的比例为 1:1:在 5 995 235 名妇女的 9 996 523 次妊娠中,发现了 1 366 例与妊娠相关的血液恶性肿瘤:其中 413 例发生在妊娠期(每 10 万例妊娠中有 4-13 例),953 例发生在妊娠结束后 12 个月内(妊娠后)(每 10 万例妊娠中有 9-53 例)。在所有类型的血液恶性肿瘤中,妊娠期血液恶性肿瘤组和妊娠后血液恶性肿瘤组的总生存率无明显差异(IPW 调整后的危险比为 0-91 [95% CI 0-62-1-34]、p=0-63),尤其是霍奇金淋巴瘤(0-56 [0-07-4-53],p=0-59)、侵袭性 B 细胞非霍奇金淋巴瘤(0-52 [0-12-2-38],p=0-40)和单纯急性白血病(0-84 [0-50-1-41],p=0-51)。与参照组相比,妊娠期间患血液恶性肿瘤组的孕产妇更容易出现严重的发病率(328 例完成妊娠中的 86 例 [26-2%] vs 7 945 909 例完成妊娠中的 120 335 例 [1-5%];IPW 调整后的几率比 22-71 [95% CI 17-72-29-10],p解释:这项全国性观察研究对法国与妊娠相关的血液恶性肿瘤进行了调查,结果显示,在妊娠期间和妊娠后确诊的妇女在总生存率方面没有明显差异。我们的数据显示,在妊娠期确诊的妇女中,严重孕产妇发病率和产科并发症的发生率有所增加。值得注意的是,这项研究强调了有效管理这些复杂病例的专业护理的必要性:无:摘要的法文译文见 "补充材料 "部分。
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引用次数: 0
Balancing the dual challenge of cancer and pregnancy: insights from large-scale data. 平衡癌症与怀孕的双重挑战:从大规模数据中获得的启示。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-10-07 DOI: 10.1016/S2352-3026(24)00308-9
Pietro R Di Ciaccio, Georgia S Mills
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引用次数: 0
Haematopoietic stem-cell transplantation for sickle cell disease in low-income and middle-income countries: the experience in India. 中低收入国家镰状细胞病的造血干细胞移植:印度的经验。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-01 DOI: 10.1016/S2352-3026(24)00272-2
Vineeta Gupta, Lakshmanan Krishnamurti
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引用次数: 0
Oh no, the light chain ratio. 哦,不,是光链比。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-10-01 DOI: 10.1016/S2352-3026(24)00310-7
Christopher Tiplady
{"title":"Oh no, the light chain ratio.","authors":"Christopher Tiplady","doi":"10.1016/S2352-3026(24)00310-7","DOIUrl":"10.1016/S2352-3026(24)00310-7","url":null,"abstract":"","PeriodicalId":48726,"journal":{"name":"Lancet Haematology","volume":" ","pages":"e814-e815"},"PeriodicalIF":15.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142376177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Sexually transmitted infections in the context of haematological malignancies. 血液恶性肿瘤中的性传播感染。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-10-01 Epub Date: 2024-09-20 DOI: 10.1016/S2352-3026(24)00210-2
Tamim Alsuliman, Paolo Musiu, Nicolas Stocker, Lana Desnica, Jean El-Cheikh, Simona Sestili, Micha Srour, Zora Marjanovic, Ali Alrstom

Sexually transmitted infections (STIs) are a difficult health challenge for immunocompromised patients. Patients treated for several haematological malignancies have further compromised immune systems. Furthermore, many chemotherapies, alone or associated with haematopoietic stem-cell transplantation, make the body's natural barriers extremely fragile. STIs can negatively impact both patient morbidity and mortality. In this Series paper, we discuss Chlamydia trachomatis, Neisseria gonorrhoeae, syphilis, human immunodeficiency virus, herpes simplex virus, human papilloma virus, and hepatitis B virus, as we found them to be associated with increased risks for haematological malignancy treatments, either by incidence or by severity. Protective measures and vaccines for patients with haematological malignancies are also discussed. Large, well conducted studies should be encouraged, with the aim to systematically analyse the impacts of STIs in patients with haematological malignancies, especially given the difficulties that antimicrobial resistance can confer to patient management.

对于免疫力低下的患者来说,性传播感染(STI)是一项棘手的健康挑战。接受多种血液恶性肿瘤治疗的患者的免疫系统会进一步受损。此外,许多化疗药物,无论是单独使用还是与造血干细胞移植联合使用,都会使人体的天然屏障变得极其脆弱。性传播感染会对患者的发病率和死亡率产生负面影响。在本系列论文中,我们将讨论沙眼衣原体、淋病奈瑟菌、梅毒、人类免疫缺陷病毒、单纯疱疹病毒、人类乳头瘤病毒和乙型肝炎病毒,因为我们发现这些病毒与血液恶性肿瘤治疗风险的增加有关,无论是发病率还是严重程度。我们还讨论了血液恶性肿瘤患者的保护措施和疫苗。应鼓励开展大规模的研究,以便系统分析性传播感染对血液恶性肿瘤患者的影响,尤其是考虑到抗菌素耐药性会给患者管理带来困难。
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引用次数: 0
Association and small risk from low-dose radiation exposure. 低剂量辐照的关联性和小风险。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-10-01 DOI: 10.1016/S2352-3026(24)00284-9
Won Jin Lee
{"title":"Association and small risk from low-dose radiation exposure.","authors":"Won Jin Lee","doi":"10.1016/S2352-3026(24)00284-9","DOIUrl":"10.1016/S2352-3026(24)00284-9","url":null,"abstract":"","PeriodicalId":48726,"journal":{"name":"Lancet Haematology","volume":"11 10","pages":"e713-e715"},"PeriodicalIF":15.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142373290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
The Lancet Haematology at 10. 柳叶刀血液学》第 10 期。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-10-01 DOI: 10.1016/S2352-3026(24)00289-8
Lan-Lan Smith, Yaiza Del Pozo Martín, Emma Cookson
{"title":"The Lancet Haematology at 10.","authors":"Lan-Lan Smith, Yaiza Del Pozo Martín, Emma Cookson","doi":"10.1016/S2352-3026(24)00289-8","DOIUrl":"10.1016/S2352-3026(24)00289-8","url":null,"abstract":"","PeriodicalId":48726,"journal":{"name":"Lancet Haematology","volume":"11 10","pages":"e709"},"PeriodicalIF":15.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142373297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Lines of the haematology community. 血液病学界的路线。
IF 15.4 1区 医学 Q1 HEMATOLOGY Pub Date : 2024-10-01 DOI: 10.1016/S2352-3026(24)00287-4
Jacqueline Del Castillo
{"title":"Lines of the haematology community.","authors":"Jacqueline Del Castillo","doi":"10.1016/S2352-3026(24)00287-4","DOIUrl":"10.1016/S2352-3026(24)00287-4","url":null,"abstract":"","PeriodicalId":48726,"journal":{"name":"Lancet Haematology","volume":"11 10","pages":"e728"},"PeriodicalIF":15.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142376179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
期刊
Lancet Haematology
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