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重度合并症骨髓纤维化患者非清髓性预处理方案后异基因造血细胞移植的经验:伴有广泛髓外造血患者的病例系列

Experiences of allogeneic hematopoietic cell transplantation following non-myeloablative conditioning regimen in severely comorbid patients with myelofibrosis: case series with a patient presenting with extensive extramedullary hematopoiesis.

作者信息

Yoon Jae-Ho, Park Sung-Soo, Min Gi June, Park Silvia, Lee Sung-Eun, Cho Byung-Sik, Kim Yoo-Jin, Lee Seok, Kim Hee-Je, Min Chang-Ki, Cho Seok-Goo, Lee Jong Wook, Eom Ki-Seong

机构信息

Department of Hematology, Catholic Hematology Hospital and Leukemia Research Institute, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Department of Hematology, Catholic Hematology Hospital and Leukemia Research Institute, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 222, Banpo-daero, Seocho-gu, Seoul 06591, Republic of Korea.

出版信息

Ther Adv Hematol. 2020 Jun 30;11:2040620720932038. doi: 10.1177/2040620720932038. eCollection 2020.

Abstract

We have performed allogeneic hematopoietic cell transplantation (allo-HCT) using a reduced intensity conditioning regimen for curative management of advanced myelofibrosis (MF). However, allo-HCT is rarely considered for elderly or patients with severe comorbidities due to high transplantation-related mortality. In those patients, an alemtuzumab-based non-myeloablative (NMA) conditioning regimen followed by stem cell transplantation could be a possible treatment that has been tried in sickle cell anemia showing stable mixed chimerism and improvement of the disease. However, it is uncertain whether this regimen can provide durable donor-dominant chimerism also in patients with MF. We planned a two-stage allo-HCT in four patients - initially aimed at mixed chimerism with NMA conditioning and then reinforced with additional stem cell infusion if graft failure occurred. In one case with extensive extramedullary hematopoiesis, causing blindness and paraplegia, we achieved stable complete donor-chimerism and complete molecular response with disappearance of bone marrow fibrosis after allo-HCT. Although this NMA regimen failed to achieve durable donor-chimerism, additional stem cell infusion showed a possible role for stable long-term chimerism with good clinical outcomes. Although it leaves room for further improvement, allo-HCT using an NMA conditioning regimen may be worth consideration for advanced MF patients with severe comorbidity, otherwise no appropriate treatment option is available.

摘要

我们采用减低强度预处理方案进行了异基因造血细胞移植(allo-HCT),用于晚期骨髓纤维化(MF)的根治性治疗。然而,由于移植相关死亡率高,老年患者或合并严重疾病的患者很少考虑进行allo-HCT。在这些患者中,基于阿仑单抗的非清髓性(NMA)预处理方案联合干细胞移植可能是一种可行的治疗方法,该方法已在镰状细胞贫血患者中尝试,显示出稳定的混合嵌合体形成及疾病改善。然而,尚不确定该方案是否也能在MF患者中提供持久的供体主导嵌合体。我们计划对4例患者进行两阶段allo-HCT——最初旨在通过NMA预处理实现混合嵌合体,若发生移植物失败则通过额外输注干细胞进行强化。在1例伴有广泛髓外造血导致失明和截瘫的患者中,allo-HCT后我们实现了稳定的完全供体嵌合体以及完全分子反应,骨髓纤维化消失。尽管这种NMA方案未能实现持久的供体嵌合体,但额外输注干细胞显示出对稳定长期嵌合体形成及良好临床结局可能发挥的作用。尽管仍有进一步改进的空间,但对于合并严重疾病的晚期MF患者,采用NMA预处理方案的allo-HCT可能值得考虑,否则没有合适的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e48/7328483/521adf4e31a3/10.1177_2040620720932038-fig1.jpg

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