Abstract:
:Shwachman-Diamond syndrome (SDS) is a rare congenital disorder featuring exocrine pancreatic insufficiency, growth retardation, and bone marrow dysfunction. Reports suggest that nearly 25% of all cases are complicated with leukemia. Although stem cell transplantation is the sole option for these patients, successful results are rarely obtained. Poor outcomes are often related to graft failure and cardiac and other organ toxicities. We describe in this report successful unrelated donor bone marrow transplantation for a patient with SDS who progressed to acute myelogenous leukemia. The patient received attenuated intensified chemotherapy because of his intolerance to ordinary chemotherapy and went into remission. Sustained unrelated donor bone marrow engraftment was accomplished after treatment with a reduced amount of cyclophosphamide and antithymocyte globulin with 12 Gy of total body irradiation as a conditioning regimen. To the best of our knowledge, this report is the first to describe unrelated donor bone marrow transplantation with complete engraftment for an SDS patient with myelogenous leukemia.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Mitsui T,Kawakami T,Sendo D,Katsuura M,Shimizu Y,Hayasaka Kdoi
10.1532/ijh97.03103subject
Has Abstractpub_date
2004-02-01 00:00:00pages
189-92issue
2eissn
0925-5710issn
1865-3774journal_volume
79pub_type
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