Pulmonary, Hepatic, and Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Telomere Biology Disorders.

IF 2.7 3区医学 Q2 HEMATOLOGY Current Hematologic Malignancy Reports Pub Date : 2024-12-01 Epub Date: 2024-02-05 DOI:10.1007/s11899-024-00724-z

Kelly M Pennington, Douglas Simonetto, Timucin Taner, Abhishek A Mangaonkar

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Abstract

Purpose of the review: This study aimed to summarize evidence and provide consensus-based guidelines for management of transplantation in patients with telomere biology disorders (TBD). Specifically, this review focuses on clinical management of lung, liver, and bone marrow transplantation in TBD patients.

Recent findings: TBD patients have specific unique biological vulnerabilities such as T cell immunodeficiency, susceptibility to infections, hypersensitivity to chemotherapy and radiation, and cytopenias. Furthermore, multiple organ involvement at diagnosis makes clinical management especially challenging due to higher degree of organ damage, and stress-induced telomeric crisis. Sequential and combined organ transplants, development of novel radiation and alkylator-free conditioning regimen, and use of novel drugs for graft-versus-host disease prophylaxis are some of the recent updates in the field. Multidisciplinary management is essential to optimize transplant outcomes in patients with TBD. In this review, we provide consensus-based transplant management guidelines for clinical management of transplant in TBD.

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端粒生物学紊乱患者的肺、肝和异体造血干细胞移植。

综述的目的：本研究旨在总结证据，为端粒生物学紊乱（TBD）患者的移植管理提供基于共识的指南。具体而言，本综述侧重于 TBD 患者肺、肝和骨髓移植的临床管理：TBD患者具有特殊的生物脆弱性，如T细胞免疫缺陷、易感染、对化疗和放疗过敏以及细胞减少症。此外，确诊时多个器官受累，由于器官损伤程度较高，加上应激引起的端粒危机，临床治疗尤其具有挑战性。序贯和联合器官移植、新型放射和无烷化剂调理方案的开发以及预防移植物抗宿主病新型药物的使用是该领域的最新进展。多学科管理对于优化 TBD 患者的移植结果至关重要。在这篇综述中，我们为 TBD 移植的临床管理提供了基于共识的移植管理指南。

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来源期刊

Current Hematologic Malignancy Reports ONCOLOGY-HEMATOLOGY

CiteScore

6.00

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： his journal intends to provide clear, insightful, balanced contributions by international experts that review the most important, recently published clinical findings related to the diagnosis, treatment, management, and prevention of hematologic malignancy. We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas, such as leukemia, lymphoma, myeloma, and T-cell and other lymphoproliferative malignancies. Section Editors, in turn, select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. An international Editorial Board reviews the annual table of contents, suggests articles of special interest to their country/region, and ensures that topics are current and include emerging research. Commentaries from well-known figures in the field are also provided.