Late death after unrelated-BMT for dyskeratosis congenita following conditioning with alemtuzumab, fludarabine and melphalan (original) (raw)

Bone Marrow Transplantation volume 40, pages 913–914 (2007)Cite this article

Brazzola and colleagues recently reported a case of fatal diffuse capillaritis after matched unrelated donor bone marrow transplant for dyskeratosis congenita (DC) despite using a minimal-intensity conditioning regimen of antithymocyte globulin (ATG), fludarabine and low-dose CY. We report a late death at 15 months post-BMT, due to gastrointestinal bleeding and obliterative fibro-alveolitis, following matched unrelated donor bone marrow transplant using alemtuzumab (campath), fludarabine and melphalan.

In 1985, the patient was diagnosed with inherited aplastic anaemia at the age of 6 years, but did not require treatment until the age of 11 years when he commenced oxymetholone with a successful response for 10 years. Family history revealed idiopathic pulmonary fibrosis (father and paternal grandmother) and his sister had non-severe aplastic anaemia. He had no evidence of Fanconi anaemia and did not display any clinical features typical of DC. Oxymetholone was discontinued in May 2003 because of abnormal liver function tests and he became transfusion-dependent.

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Authors and Affiliations

  1. Department of Haematology, St George's Hospital, London, UK
    K Amarasinghe, C Dalley, V Gupta & J Marsh
  2. Academic Department of Paediatrics, Institute of Cell and Molecular Science, Barts and The London Hospital, London, UK
    I Dokal
  3. Department of Haematology, Ashford and St Peter's Hospital, Middlesex, UK
    A Laurie
  4. Department of Cellular and Molecular Medicine, St George's University of London, London, UK
    J Marsh

Authors

  1. K Amarasinghe
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  2. C Dalley
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  3. I Dokal
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  4. A Laurie
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  5. V Gupta
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  6. J Marsh
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Correspondence toJ Marsh.

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Amarasinghe, K., Dalley, C., Dokal, I. et al. Late death after unrelated-BMT for dyskeratosis congenita following conditioning with alemtuzumab, fludarabine and melphalan.Bone Marrow Transplant 40, 913–914 (2007). https://doi.org/10.1038/sj.bmt.1705839

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