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Successful combined venetoclax to PD-1 blockade and ruxolitinib for refractory Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis. Venetoclax联合PD-1阻断剂和Ruxolitinib治疗难治性Epstein-Barr病毒相关性嗜血细胞淋巴组织细胞增多症获得成功。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-09 DOI: 10.1111/bjh.19900
Yue Song, Xiaofei Yang, Qian Wu, Mengxing Xue, Fei Zhou, Depei Wu, Suning Chen, Xuefeng He
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引用次数: 0
Chronic hepatitis E in a patient after chimeric antigen receptor-T-cell treatment for diffuse large B-cell lymphoma and rapid progression towards decompensated liver cirrhosis. 一名弥漫大 B 细胞淋巴瘤患者在接受嵌合抗原受体-T 细胞治疗后出现慢性戊型肝炎,并迅速发展为肝硬化失代偿期。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-07 DOI: 10.1111/bjh.19892
Michael Schwarz, Behrang Mozayani, Michael Trauner, Albert Friedrich Stättermayer
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引用次数: 0
Long-term follow-up of bulky classic Hodgkin lymphoma managed with ABVD and PET-guided RT demonstrates excellent outcomes in PET-negative cases. 对采用 ABVD 和 PET 引导 RT 治疗的巨大典型霍奇金淋巴瘤进行的长期随访显示,PET 阴性病例的疗效极佳。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-07 DOI: 10.1111/bjh.19859
J L Kim, D Villa, R P Tonseth, A S Gerrie, D Wilson, F Benard, C P Venner, B Skinnider, P Farinha, G W Slack, A C Lo, D W Scott, L H Sehn, K J Savage

The outcome of 221 patients with bulky (≥10 cm) classic Hodgkin lymphoma (cHL) treated with doxorubicin, bleomycin, vinblastine, dacarbazine and consolidative radiotherapy (RT) only in those with a positive end-of-treatment (EOT) positron emission tomography (PET) scan was evaluated. With a median follow-up of 9.6 years, 5- and 10-year progression-free survival (PFS) in EOT PET-negative cases were 94.0% and 90.4%, respectively, and in PET-positive cases, 5/10-year PFS was 64.6% (p < 0.001), with 15% overall receiving RT. Five-year PFS for Deauville (D) score DX/D1-3 was 93.6%, compared with D4 66.7% (p < 0.001) and D5 33.3% (p < 0.001). Omission of RT in EOT PET-negative cases is associated with excellent long-term outcomes in bulky cHL.

该研究评估了221例肿块型(≥10厘米)典型霍奇金淋巴瘤(cHL)患者的疗效,这些患者均接受过多柔比星、博来霉素、长春新碱、达卡巴嗪和综合放疗(RT)治疗,只有治疗末期(EOT)正电子发射断层扫描(PET)扫描结果呈阳性。中位随访 9.6 年,EOT PET 阴性病例的 5 年和 10 年无进展生存率(PFS)分别为 94.0% 和 90.4%,PET 阳性病例的 5/10 年无进展生存率为 64.6%(P<0.05)。
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引用次数: 0
Delaying pegaspargase during induction in adults with acute lymphoblastic leukaemia is associated with lower risk of high-grade hepatotoxicity without adversely impacting outcomes. 在急性淋巴细胞白血病成人患者的诱导治疗过程中延迟使用培加司琼酶可降低高度肝毒性的风险,同时不会对治疗效果产生不利影响。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-06 DOI: 10.1111/bjh.19880
Jose Tinajero, Sharon Xu, Dat Ngo, Shanpeng Li, Joycelynne Palmer, Tina Nguyen, Anthony Stein, Paul Koller, Vaibhav Agrawal, Hoda Pourhassan, Lindsey Murphy, Stephen Forman, Dan Douer, Guido Marcucci, Vinod Pullarkat, Ibrahim Aldoss

Pegaspargase is a key drug for the treatment of younger adults with acute lymphoblastic leukaemia (ALL). Pegaspargase-associated hepatotoxicity is most common during induction, and its incidence increases with age and body mass index (BMI). We hypothesized that the delayed administration of pegaspargase during induction is associated with lower risk of hepatotoxicity while retaining efficacy. We retrospectively reviewed 141 adult patients with newly diagnosed ALL who received pegaspargase during induction from November 2013 to February 2024. There were 78 (55.3%) patients who received early pegaspargase (EP) on day 4 and 63 (44.7%) patients who received delayed pegaspargase (DP) on day 15. High-grade hepatotoxicity (grade ≥ 3 transaminitis and/or hyperbilirubinaemia) occurred more frequently in the EP group (p = 0.06). Rates of complete remission and negative minimal residual disease post induction were not different between cohorts. Univariate logistic regression analysis showed that BMI and age significantly predicted an increased risk of high-grade hepatotoxicity while DP was associated with a lower risk (odds ratio = 0.44; p = 0.04). Overall survival and event-free survival were not significantly different between cohorts. Delaying pegaspargase administration from day 4 to day 15 during induction cycle in adults with ALL might reduce the risk of high-grade hepatotoxicity without adversely impacting clinical efficacy outcomes.

培加司琼酶是治疗年轻成人急性淋巴细胞白血病(ALL)的主要药物。与培加司琼酶相关的肝毒性在诱导期间最为常见,其发生率随年龄和体重指数(BMI)的增加而增加。我们假设,在诱导期间延迟使用培加司琼酶可降低肝毒性风险,同时保持疗效。我们回顾性研究了2013年11月至2024年2月期间新诊断为ALL的141例成人患者,这些患者在诱导期间接受了培加司通治疗。其中78例(55.3%)患者在第4天接受了早期培加司的(EP)治疗,63例(44.7%)患者在第15天接受了延迟培加司的(DP)治疗。高级别肝毒性(转氨酶≥3级和/或高胆红素血症)在EP组发生率更高(p = 0.06)。诱导后完全缓解率和阴性极小残留病率在各组间没有差异。单变量逻辑回归分析表明,体重指数和年龄可显著预测高级别肝毒性风险的增加,而DP与较低的风险相关(几率比=0.44;P=0.04)。各组间的总生存期和无事件生存期无明显差异。在成人 ALL 诱导周期中,将 pegaspargase 的给药时间从第 4 天推迟到第 15 天可能会降低高级别肝毒性的风险,而不会对临床疗效产生不利影响。
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引用次数: 0
Hereditary pyropoikilocytosis resulting in artefactual thrombocytosis. 遗传性热核细胞增多症导致假性血小板增多。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-06 DOI: 10.1111/bjh.19868
Maria-Angustias Molina-Arrebola, Maria Morales-Pineda, Barbara J Bain
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引用次数: 0
Clinical outcomes in patients in any phase of CML treated with ponatinib in France-Data from the TOPASE observational study. 法国使用泊纳替尼治疗任何阶段CML患者的临床结果--来自TOPASE观察性研究的数据。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-06 DOI: 10.1111/bjh.19819
F Huguet, A Guerci-Bresler, G Roth-Guepin, E Cayssials, B Slama, A Santagostino, A Penot, P Quittet, P Cony-Makhoul, A Saad, J N Bastie, M Hacini, V Coiteux, M Uzunov, L Roy, L Le Clech, M Berger, A M Agneray, E Messas, G Etienne, A Turhan, F E Nicolini, P Rousselot

The TOPASE study was set up to evaluate the outcomes of chronic myeloid leukaemia [CML] patients treated with ponatinib (PON) in a real-world setting in France. One hundred and twenty CML patients, 105 in chronic phase (CP), 8 in accelerated phase (AP) and 7 in blastic phase (BP) were included. Fifty-one (49%) of the CP-CML patients were in third line of treatment. The trigger for PON initiation in CP-CML was 'poor response' in 67 patients, 'poor tolerance' in 28 patients and 'response enhancement' in seven patients. The median dose at initiation was 30 mg/day [Q1; Q3 = 15; 30] in CP-CML and 45 mg/day [Q1; Q3 = 30; 45] in AP/BP-CML. Of 98 CP-CML evaluable patients, 72 (73.5%) were considered as responders (MMR) at one time point at least once, especially for those in second line of treatment and/or presenting a T315I mutation. Ninety-six of 120 (80%) patients reported at least one adverse event. An arterial occlusive event (AOE) was reported in 11 patients (9.2%). Thus, these real-life data confirm the potency of ponatinib in resistant or intolerant patients with an acceptable safety profile in non-selected patients. NCT number: NCT04048564.

TOPASE研究旨在评估在法国实际环境中接受泊纳替尼(PON)治疗的慢性髓性白血病(CML)患者的疗效。该研究共纳入120名CML患者,其中105名处于慢性期(CP),8名处于加速期(AP),7名处于分裂期(BP)。在 CP-CML 患者中,有 51 人(49%)处于三线治疗阶段。在 CP-CML 患者中,67 名患者开始使用 PON 的诱因是 "反应不佳",28 名患者是 "耐受性差",7 名患者是 "反应增强"。CP-CML患者开始治疗时的中位剂量为30毫克/天[Q1; Q3 = 15; 30],AP/BP-CML患者为45毫克/天[Q1; Q3 = 30; 45]。在98例可评估的CP-CML患者中,72例(73.5%)至少有一次在某个时间点被视为应答者(MMR),尤其是那些处于二线治疗和/或出现T315I突变的患者。120名患者中有96名(80%)报告了至少一次不良事件。11名患者(9.2%)报告了动脉闭塞事件(AOE)。因此,这些现实生活中的数据证实了泊纳替尼对耐药或不耐药患者的疗效,以及对非选择性患者可接受的安全性。NCT 编号:NCT04048564:NCT04048564。
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引用次数: 0
Editor's Note to the letter 'Therapeutic potential of the latest oxygen affinity modifying agent, GBT021601, for treating sickle cell disease is questionable' by Drs. Eaton, Alaimo, Metaferia, Cellmer, Thein and Bunn. 编者按:伊顿博士、阿莱莫博士、梅塔费利亚博士、塞尔默博士、西恩博士和布恩博士的来信《最新氧亲和力修饰剂 GBT021601 治疗镰状细胞病的潜力值得怀疑》。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-06 DOI: 10.1111/bjh.19865
Peter Hokland
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引用次数: 0
Relevance of the E756del common variant in the PIEZO1 gene for haemolytic anaemia and hepatic iron overload. PIEZO1 基因中的 E756del 常见变异与溶血性贫血和肝铁超负荷的相关性。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-06 DOI: 10.1111/bjh.19886
Federica Maria Esposito, Vanessa D'Onofrio, Barbara Eleni Rosato, Roberta Marra, Antonella Nostroso, Anthony Iscaro, Mariangela Manno, Michela Ribersani, Virginia Giorgi, Renata Celia, Carmelo Piscopo, Achille Iolascon, Roberta Russo, Immacolata Andolfo
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引用次数: 0
Therapeutic potential of the latest oxygen affinity-modifying agent, GBT021601, for treating sickle cell disease is questionable. 最新的氧亲和力修饰剂 GBT021601 治疗镰状细胞病的潜力值得怀疑。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-06 DOI: 10.1111/bjh.19697
Braydon Alaimo, Belhu Metaferia, Troy Cellmer, Swee Lay Thein, H Franklin Bunn, William A Eaton
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引用次数: 0
Assessment of fitness for bleomycin use and management of bleomycin pulmonary toxicity in patients with classical Hodgkin lymphoma: A British Society for Haematology Good Practice Paper. 经典霍奇金淋巴瘤患者是否适合使用博莱霉素以及博莱霉素肺毒性的处理:英国血液学会良好实践论文。
IF 5.1 2区 医学 Q1 HEMATOLOGY Pub Date : 2024-11-06 DOI: 10.1111/bjh.19840
Aisling Barrett, Nimish Shah, Andrew Chadwick, David Burns, Cathy Burton, David J Cutter, George A Follows, Pam McKay, Wendy Osborne, Elizabeth Phillips, Matthew R Wilson, Graham P Collins

This good practice paper (GPP) is intended to support clinicians in assessing patient fitness for bleomycin and in management of bleomycin pulmonary toxicity (BPT) where it occurs. Bleomycin, originally developed as an antibiotic in the 1960s, has been a cornerstone of therapy for classical Hodgkin lymphoma (CHL) since results of its use in combination with doxorubicin, vincristine and dacarbazine (ABVD) were first published by Bonadonna et al in 1975 1. The same author recognised high rates of respiratory morbidity in these patients 2, and bleomycin-;related pulmonary toxicity (BPT) is now a well-;recognised and feared complication with its use. ABVD and BEACOPP/ BEACOPDac (bleomycin, cyclophosphamide, etoposide, doxorubicin, vincristine and prednisolone, with procarbazine or dacarbazine) are standard first-;line treatments in CHL patients, but considerable variation remains in assessing patient fitness for bleomycin both clinically and with respiratory investigations. A recent survey of British haematologists regularly using bleomycin revealed that 87.5% have no local protocols for assessing patients in an evidence-;based fashion, with wide variations in practice captured in the same survey (personal data). A working group was established and a literature review undertaken with the goal of presenting practical recommendations for clinicians regarding bleomycin use based on available evidence and expert opinion.

本良好实践文件(GPP)旨在帮助临床医生评估患者是否适合使用博莱霉素,以及如何处理博莱霉素肺毒性(BPT)。博莱霉素最初是在20世纪60年代作为一种抗生素开发出来的,自1975年Bonadonna等人首次发表博莱霉素与多柔比星、长春新碱和达卡巴嗪(ABVD)联合使用的结果以来,博莱霉素一直是治疗典型霍奇金淋巴瘤(CHL)的基石1。 同一位作者指出,这些患者的呼吸系统发病率很高2,博莱霉素相关肺毒性(BPT)现已成为一种公认的、令人担忧的并发症。ABVD和BEACOPP/ BEACOPDac(博来霉素、环磷酰胺、依托泊苷、多柔比星、长春新碱和泼尼松龙,加丙卡巴嗪或达卡巴嗪)是CHL患者的标准一线治疗方法,但在临床和呼吸系统检查中,对患者是否适合使用博来霉素的评估仍存在相当大的差异。最近对经常使用博莱霉素的英国血液学家进行的一项调查显示,87.5%的英国血液学家没有以循证医学为基础的评估患者的本地方案,而且在同一调查(个人数据)中还发现了很大的实践差异。我们成立了一个工作小组,并进行了文献综述,目的是根据现有证据和专家意见,为临床医生提供使用博来霉素的实用建议。
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British Journal of Haematology
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