Department of Haematology, Guy's and St. Thomas' NHS Foundation Trust and University College Hospitals, London, UK.
Hospital Clinico Universitario, Valencia, Spain.
Leukemia. 2021 Sep;35(9):2445-2459. doi: 10.1038/s41375-021-01294-2. Epub 2021 May 26.
Allogeneic haematopoietic cell transplantation (allo-HCT) remains the only curative approach in myelofibrosis (MF). Despite advances over recent decades, relapse and non-relapse mortality rates remain significant. Relapse rates vary between 15 and 25% across retrospective studies and management strategies vary widely, ranging from palliation to adoptive immunotherapy and, in some cases, a second allo-HCT. Moreover, in allo-HCT, there is a higher incidence of poor graft function and graft failure due to splenomegaly and a hostile "pro-inflammatory" marrow niche. The Practice Harmonisation and Guidelines subcommittee of the Chronic Malignancies Working Party (CMWP) of EBMT convened an international panel consisting of transplant haematologists, histopathologists and molecular biologists to propose practical, clinically relevant definitions of graft failure, poor graft function and relapse as well as management strategies following allo-HCT. A systematic approach to molecular monitoring, histopathological assessment and chimerism testing is proposed. These proposed recommendations aim to increase the accuracy and uniformity of reporting and to thereby facilitate the development of more consistent approaches to these challenging issues. In addition, we propose management strategies for these complications.
异基因造血细胞移植(allo-HCT)仍然是骨髓纤维化(MF)的唯一治愈方法。尽管近几十年来取得了进展,但复发和非复发死亡率仍然很高。在回顾性研究中,复发率在 15%至 25%之间变化,管理策略差异很大,从姑息治疗到过继免疫治疗,在某些情况下还需要进行第二次 allo-HCT。此外,在 allo-HCT 中,由于脾肿大和敌对的“促炎”骨髓微环境,嵌合率低和移植物功能不良的发生率更高。EBMT 的慢性恶性肿瘤工作组(CMWP)的实践协调和指南小组委员会召集了一个由移植血液学家、组织病理学家和分子生物学家组成的国际小组,提出了实用的、与临床相关的嵌合率低、移植物功能不良和复发的定义以及 allo-HCT 后的管理策略。提出了一种系统的分子监测、组织病理学评估和嵌合体检测方法。这些建议旨在提高报告的准确性和一致性,从而促进对这些具有挑战性问题的更一致方法的发展。此外,我们还提出了这些并发症的管理策略。