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Post-Transplant Complications

Life-threatening pulmonary hemorrhages post bone marrow transplantation in Hurler syndrome. Report of three cases and review of the literature

Bone Marrow Transplantation volume 32, pages 213–215 (2003)Cite this article

Summary:

Hurler syndrome (MPS-IH) is an autosomal recessive mucopolysaccharide storage disorder caused by deficiency of lysosomal alpha-L-iduronidase (IDU) enzyme activity. This results in accumulation of heparan sulfate and dermatan sulfate substances. Untreated children develop progressive developmental deterioration and multisystem morbidity with a median survival of 5 years. Allogeneic bone marrow transplantation (BMT) is the only long-lasting treatment that ameliorates or halts the aggressive course of the disease. Pulmonary hemorrhage (PH) is an unusual complication of BMT and has not been previously reported in MPS-IH post-BMT. We report three children with MPS-IH with life-threatening PH around the time of engraftment. All needed intensive-care support and one child developed recurrent PH that required prolonged ventilation.

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Acknowledgements

We are grateful to Kim Yule, Christine Armstrong, BMT, PICU and metabolic staff and nurses for their excellent technical assistance.

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  1. Divisions of Hematology/Oncology and Clinical/metabolic Genetics, The Hospital for Sick Children, University of Toronto, Toronto, Ont., Canada

    A Gassas, L Sung, J J Doyle, J T R Clarke & E Fred Saunders

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  1. A Gassas
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  2. L Sung
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  3. J J Doyle
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  4. J T R Clarke
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  5. E Fred Saunders
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Gassas, A., Sung, L., Doyle, J. et al. Life-threatening pulmonary hemorrhages post bone marrow transplantation in Hurler syndrome. Report of three cases and review of the literature. Bone Marrow Transplant 32, 213–215 (2003). https://doi.org/10.1038/sj.bmt.1704115

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  • DOI: https://doi.org/10.1038/sj.bmt.1704115

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