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基于白消安的清髓性预处理后极年幼患儿脐带血移植的远期效应

Late Effects after Umbilical Cord Blood Transplantation in Very Young Children after Busulfan-Based, Myeloablative Conditioning.

作者信息

Allewelt Heather, El-Khorazaty Jill, Mendizabal Adam, Taskindoust Mahsa, Martin Paul L, Prasad Vinod, Page Kristin, Sanders Jean, Kurtzberg Joanne

机构信息

Department of Pediatrics, Pediatric Blood and Marrow Transplant Program, Duke University Medical Center, Durham, North Carolina.

The EMMES Corporation, Rockville, Maryland.

出版信息

Biol Blood Marrow Transplant. 2016 Sep;22(9):1627-1635. doi: 10.1016/j.bbmt.2016.05.024. Epub 2016 Jun 2.

Abstract

Infants and young children who undergo allogeneic cord blood transplantation (CBT) are at increased risk for late effects because of exposure of developing organs to chemotherapy and radiation therapy typically used in transplant conditioning regimens. Busulfan (Bu)-based myeloablative regimens were developed to eliminate radiation exposure in these young children with the hope that late effects would be minimized. We now describe the late effects in 102 consecutive patients surviving a minimum of 5 years (median follow-up, 12.9 years) post-CBT. Patients were conditioned with high-dose chemotherapy using Bu-containing regimens. No patient received total body irradiation. The median age at transplant was 1 year (range, .1 to 2). Diagnoses included inherited metabolic diseases (59.8%), leukemia (17.6%), congenital immune deficiency (20.2%), bone marrow failure/myelodysplastic syndrome (3.9%), and hemoglobinopathy (2%). Among patients surviving 5 years, the overall survival rate at 10 years post-CBT was 93% (95% CI, 84.9 to 96.8). Virtually all patients (98%) experienced at least 1 significant late effect. Most (83.3%) experienced 2 or more late effects, and more than half of the patients (64.7%) experienced 3 or more late effects. The most commonly observed late effects included dental problems (92.2%), short stature (55.9%), cognitive deficits (53.6%), pulmonary dysfunction (18.6%), and abnormal pubertal development (27.9%). This is the first report of late effects of Bu-based conditioning in a cohort of very young patients at the time of transplant. These results will inform clinical care guidelines for long-term follow-up and add to the growing information regarding outcomes of hematopoietic stem cell transplantation.

摘要

接受异基因脐血移植(CBT)的婴幼儿因发育中的器官暴露于移植预处理方案中常用的化疗和放射治疗而面临更高的迟发效应风险。基于白消安(Bu)的清髓方案旨在消除这些幼儿的辐射暴露,希望将迟发效应降至最低。我们现在描述了102例CBT后存活至少5年(中位随访时间为12.9年)的连续患者的迟发效应。患者接受含Bu方案的大剂量化疗进行预处理。没有患者接受全身照射。移植时的中位年龄为1岁(范围为0.1至2岁)。诊断包括遗传性代谢疾病(59.8%)、白血病(17.6%)、先天性免疫缺陷(20.2%)、骨髓衰竭/骨髓增生异常综合征(3.9%)和血红蛋白病(2%)。在存活5年的患者中,CBT后10年的总生存率为93%(95%CI,84.9至96.8)。几乎所有患者(98%)都经历了至少1种显著的迟发效应。大多数(83.3%)经历了2种或更多种迟发效应,超过一半的患者(64.7%)经历了3种或更多种迟发效应。最常观察到的迟发效应包括牙齿问题(92.2%)、身材矮小(55.9%)、认知缺陷(53.6%)、肺功能障碍(18.6%)和青春期发育异常(27.9%)。这是关于移植时非常年幼患者队列中基于Bu预处理的迟发效应的首次报告。这些结果将为长期随访的临床护理指南提供参考,并增加有关造血干细胞移植结果的信息。

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