Document Detail

Reduced-intensity conditioning is effective and safe for transplantation of patients with Shwachman-Diamond syndrome.
MedLine Citation:
PMID:  18500373     Owner:  NLM     Status:  MEDLINE    
Allogeneic hematopoietic stem cell transplantation (HSCT) is the only potentially curative treatment for the BM dysfunction seen in patients with Shwachman-Diamond syndrome (SDS). Historically, these patients have fared poorly with intensive conditioning regimens with increased regimen-related toxicity especially involving the heart and lungs. We report our institutional experience with a reduced-intensity-conditioning protocol in seven patients with SDS and BM aplasia or myelodysplastic syndrome/AML. The preparative regimen consisted of Campath-1H, fludarabine and melphalan. Four patients received matched related marrow and three received unrelated stem cells (two PBSCs and one marrow). All but one was 8 of 8 allele HLA matched. All patients established 100% donor-derived hematopoiesis. No patient in this cohort developed grades III-IV GVHD. One patient had grade II skin GVHD that responded to systemic corticosteroids and one had grade I skin GVHD, treated with topical corticosteroids. Two out of seven patients developed bacterial infections in the early post transplant period. Viral infections were seen in four out of seven patients and were successfully treated with appropriate antiviral therapy. All patients are currently alive. These data indicate that HSCT with reduced-intensity conditioning is feasible in patients with SDS and associated with excellent donor cell engraftment and modest morbidity.
D Bhatla; S M Davies; S Shenoy; R E Harris; M Crockett; L Shoultz; T Smolarek; J Bleesing; M Hansen; S Jodele; M Jordan; A H Filipovich; P A Mehta
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Publication Detail:
Type:  Journal Article     Date:  2008-05-26
Journal Detail:
Title:  Bone marrow transplantation     Volume:  42     ISSN:  0268-3369     ISO Abbreviation:  Bone Marrow Transplant.     Publication Date:  2008 Aug 
Date Detail:
Created Date:  2008-08-13     Completed Date:  2008-11-13     Revised Date:  2013-05-27    
Medline Journal Info:
Nlm Unique ID:  8702459     Medline TA:  Bone Marrow Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  159-65     Citation Subset:  IM    
Department of Hematology Oncology, Cincinnati Children's Hospital and Medical Center, Cincinnati, OH, USA.
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MeSH Terms
Abnormalities, Multiple / surgery*
Antibodies, Monoclonal / therapeutic use
Antibodies, Monoclonal, Humanized
Antibodies, Neoplasm / therapeutic use
Child, Preschool
Drug Therapy, Combination
Graft vs Host Disease / prevention & control
Melphalan / therapeutic use
Pancreatic Diseases / surgery*
Stem Cell Transplantation*
Transplantation Conditioning / adverse effects,  methods*
Vidarabine / analogs & derivatives,  therapeutic use
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Antibodies, Monoclonal, Humanized; 0/Antibodies, Neoplasm; 148-82-3/Melphalan; 3A189DH42V/alemtuzumab; 5536-17-4/Vidarabine; P2K93U8740/fludarabine

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