PMID: 8597612Feb 1, 1996Paper

Allogeneic bone marrow transplatation in a patient with Shwachman-Diamond syndrome

Annals of Hematology
L ArsenievH Link

Abstract

Shwachman-Diamond syndrome (SDS) is a rare inherited disorder involving concomitant neutropenia and exocrine pancreatic insufficiency. About 25% of patients develop hematopoietic malignancies. We describe a 24-year-old male patient with SDS who underwent allogeneic bone marrow transplantation (BMT) because of progression into acute myeloid leukemia (AML) following myelodysplastic syndrome (MDS). The BMT preparative regimen consisted of busulfan (16 mg/kg body wt.), followed by cyclophosphamide (120 mg/kg). Cyclosporin A and short methotrexate were used for graft-versus-host disease (GvHD) prophylaxis. The post-transplant period was complicated by staphylococcal septicemia, CMV infection, renal insufficiency, and acute GvHD grade III. Hematological recovery was delayed (post-transplant day +55). The patient was discharged at day +68 in complete remission without any evidence of MDS. RFLP fingerprint analysis showed complete engraftment of the donor's hematopoiesis. The patient's leukemia relapsed 9 months post-transplant, and death followed due to CMV infection and multiorgan failure. Despite the fatal course in this patient, allogeneic BMT could be an option for curative treatment of the hematopoietic failure in SDS. The intera...Continue Reading

Citations

Dec 28, 2011·Musculoskeletal Surgery·C Dall'ocaP Bartolozzi
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Aug 16, 2002·British Journal of Haematology·Yigal Dror, Melvin H Freedman
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Apr 12, 2012·Haematologica·Jean DonadieuUNKNOWN Associated investigators of the French Severe Chronic Neutropenia Registry*
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