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Bone marrow transplantation in Shwachman–Diamond syndrome

Bone Marrow Transplantation volume 30, pages 255–258 (2002)Cite this article

Abstract

Shwachman–Diamond syndrome is a rare autosomal recessive disorder characterized by exocrine pancreatic dysfunction, metaphyseal dysostosis and bone marrow dysfunction with a predilection towards severe hematologic complications. Allogeneic bone marrow transplantation has been used as a theraputic approach for SDS patients with serious hematologic abnormalities with mixed results. There is some concern that these patients may be more suceptible to early (<100 days) transplant-related complications than other transplant groups. We report a patient who received a matched allogeneic transplant without developing serious early transplant-related complications, but eventually died from relapse of his disease. Although experience is limited, a review of the reported cases suggests patients with SDS may be transplanted without significant short-term morbidity and mortality.

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Acknowledgements

Robert A Brodsky is a Leukemia and Lymphoma Society Clinical Research Scholar.

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  1. Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD, USA

    JW Hsu, G Vogelsang, RJ Jones & RA Brodsky

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  1. JW Hsu
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  2. G Vogelsang
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Hsu, J., Vogelsang, G., Jones, R. et al. Bone marrow transplantation in Shwachman–Diamond syndrome. Bone Marrow Transplant 30, 255–258 (2002). https://doi.org/10.1038/sj.bmt.1703631

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