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

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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.

Summary

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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Data Availability

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Acknowledgements

The authors would like to thank the patients and family members belonging to the telomere biology disorder community.

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Authors

Contributions

KP wrote the lung transplantation section, and DS and TT wrote the liver transplantation section. AM wrote the bone marrow transplantation and other sections of the manuscript. All authors contributed equally.

Corresponding authors

Correspondence to Kelly M. Pennington, Douglas Simonetto, Timucin Taner or Abhishek A. Mangaonkar.

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Pennington, K.M., Simonetto, D., Taner, T. et al. Pulmonary, Hepatic, and Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Telomere Biology Disorders. Curr Hematol Malig Rep (2024). https://doi.org/10.1007/s11899-024-00724-z

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