东地中海地区急性和慢性移植物抗宿主病的治疗和管理:全球血液和骨髓移植网络调查

Ibrahim N Muhsen, Dietger Niederwieser, Laurent Garderet, Olaf Penack, Hildegard T Greinix, Riad El Fakih, Nour Ben Abdeljelil, Ibraheem Abosoudah, Sameer Alamoudi, Amal Albeihany, Saad Ahmed Al Daama, Mohammad Hamad Alshahrani, Salem Alshemmari, Murtadha Al-Khabori, Ahlam Almasari, Abdulhakim Al Rawas, Medhat Askar, Ali Bazarbachi, Mohammed-Amine Bekadja, Malek Benakli, Munira Borhany, Maria El Kababri, Khalid Halahleh, Amir Ali Hamidieh, Mahmoud Hammad, Ahmad Ibrahim, Solaf Kanfar, Mohamed Hamed Khalaf, Mohammed Marei, Muhammad Ayaz Mir, Dania Monagel, Asma Quessar, Rawad Rihani, Munira Shabbir-Moosajee, Marwan Shaheen, Almetwaly Mohamed Sultan, Mohammad Vaezi, Damiano Rondelli, Mickey Boon Chai Koh, Zina Peric, Yoshiko Atsuta, Naeem Chaudhri, Mahmoud Aljurf
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引用次数: 0

摘要

背景:急性和慢性移植物抗宿主病(GvHD)的治疗仍然是一个挑战,特别是在类固醇难治性GvHD的情况下。GvHD的管理因机构而异,对世界不同区域的做法知之甚少。因此,世界血液和骨髓移植网络制定了一份调查问卷,以了解目前东地中海(EM)地区GvHD管理的做法。方法:问卷共有46项,并以电子方式分发给新兴市场地区的移植中心。收到的答复是在2022年12月至2023年6月之间。调查问卷针对急性和慢性GvHD的管理,包括新诊断和难治性病例。结果:调查问卷由分布在11个国家的26所院校的30个项目完成。对于新诊断的急性GvHD患者,大多数项目报道使用全身类固醇作为初始治疗,剂量根据病情的严重程度选择:IIa级相当于1mg /kg/天强的松,IIb级相当于2mg /kg/天。除类固醇外,大多数项目继续免疫抑制治疗,如果GvHD在停止后发展,则重新引入免疫抑制治疗。对于类固醇难治性患者,ruxolitinib是最常选择的二线治疗,80%的方案选择,其次是钙调磷酸酶抑制剂(47%),高剂量类固醇(bbb20 2 mg/kg, 43%),霉酚酸酯(MMF, 40%)和体外光化学(ECP, 40%)。另一方面,对于新诊断的慢性GvHD患者,全身性类固醇用于局部治疗无法获得的轻度慢性GvHD和中至重度疾病的初始治疗,最常用的初始剂量分别相当于0.5至1和bbb1mg /kg/天的强的松。超过三分之二的项目在停止免疫抑制治疗后发展为中度/重度慢性GvHD的患者中使用类固醇以外的另一种药物。对于类固醇难治性慢性GvHD患者,大多数方案在二线设置中选择多种选择,最常见的选择是鲁索利替尼(77%),钙调磷酸酶抑制剂(68%),MMF(53%),伊马替尼(53%),ECP(50%),利妥昔单抗(47%)和伊鲁替尼(40%)。结论:我们的研究结果表明,新兴市场地区的GvHD管理实践总体上符合当前的指导方针。然而,研究结果强调,获得临床试验和多学科支持团队的机会仍然有限。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acute and chronic graft-versus-host disease treatment and management in the Eastern Mediterranean region: A Worldwide Network for Blood and Marrow Transplantation survey.

Background: The treatment of acute and chronic graft-versus-host disease (GvHD) remains a challenge, particularly in cases of steroid-refractory GvHD. The management of GvHD varies between institutions, and little is known regarding the practices in different regions of the world. Thus, the Worldwide Network for Blood and Marrow Transplantation has developed a questionnaire to understand the current practices of GvHD management in the Eastern Mediterranean (EM) region.

Methodology: The questionnaire had 46 items and was distributed electronically to transplant centers in the EM region. Responses were received between December 2022 and June 2023. The questionnaire addressed the management of acute and chronic GvHD for both newly diagnosed and refractory cases.

Results: The questionnaire was completed by 30 programs across 26 institutions located in 11 countries. For patients with newly diagnosed acute GvHD, most programs reported the use of systemic steroids for initial treatment, with doses selected based on the severity of the presentation: the equivalent of 1 mg/kg/day of prednisone for grade IIa and 2 mg/kg/day for grade IIb. In addition to steroids, most programs continued immunosuppressive therapy or reintroduced it if GvHD developed after its cessation. For patients who were refractory to steroids, ruxolitinib was the most frequently selected second-line treatment, chosen by 80% of the programs, followed by calcineurin inhibitors (47%), high-dose steroids (>2 mg/kg, 43%), mycophenolate mofetil (MMF, 40%), and extracorporeal photopheresis (ECP, 40%). On the other hand, for patients with newly diagnosed chronic GvHD, systemic steroids are used for the initial management of mild chronic GvHD not accessible by topical treatment and moderate to severe disease, with the most commonly used initial dose being the equivalent of 0.5 to 1 and >1 mg/kg/day of prednisone, respectively. More than two-thirds of the programs use another agent in addition to steroids in patients who develop moderate/severe chronic GvHD while off immunosuppressive therapy. For patients with steroid-refractory chronic GvHD, most programs selected multiple options in the second-line setting, with the most frequently selected options being ruxolitinib (77%), calcineurin inhibitors (68%), MMF (53%), imatinib (53%), ECP (50%), rituximab (47%), and ibrutinib (40%).

Conclusion: Our results demonstrated that GvHD management practices in the EM region generally align with current guidelines. However, the results highlight that access to clinical trials and multidisciplinary support teams remains limited.

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