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.
Original language | English (US) |
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Pages (from-to) | 255-258 |
Number of pages | 4 |
Journal | Bone marrow transplantation |
Volume | 30 |
Issue number | 4 |
DOIs | |
State | Published - 2002 |
Keywords
- Allogeneic transplant
- Aplastic anemia
- Congenital anemia
- Myelodyplasia
- Pancreatic insufficiency
- Shwachman-Diamond syndrome
ASJC Scopus subject areas
- Hematology
- Transplantation