Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Letter to the Editor
  • Published:

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

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.

This is a preview of subscription content, access via your institution

Relevant articles

Open Access articles citing this article.

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

References

  1. Vulliamy TJ, Marrone A, Knight SW, Walne A, Mason PJ, Dokal I . Mutations in dyskeratosis congenita: their impact on telomere length and the diversity of clinical presentation. Blood 2006; 107: 2680–2685.

    Article  CAS  PubMed  Google Scholar 

  2. Marrone A, Sokhal P, Walne A, Beswick R, Kirwan M, Killick S et al. Functional characterisation of novel telomerase RNA (TERC) mutations in patients with diverse clinical and pathological presentations. Haematologica 2007; 92: 1013–1020.

    Article  CAS  PubMed  Google Scholar 

  3. Dokal I . Dyskeratosis congenita in all its forms. Br J Haematol 2000; 110: 768–779.

    Article  CAS  PubMed  Google Scholar 

  4. Nobili B, Rossi G, DeStefano P, Zecca M, Giorgiani G, Perrotta S et al. Successful umbilical cord blood transplantation in a child with dyskeratosis congenita after a fludarabine-based reduced-intensity conditioning regimen. Br J Haematol 2002; 119: 573–574.

    Article  PubMed  Google Scholar 

  5. Gungor T, Corbacioglu S, Storb R, Seger RA . Nonmyeloablative allogeneic haematopoietic stem cell transplantation for treatment of dyskeratosis congenita. Bone Marrow Transplant 2003; 31: 407–410.

    Article  CAS  PubMed  Google Scholar 

  6. Dror Y, Freedman MH, Leaker M, Verbeek J, Armstrong CA, Saunders FE et al. Low intensity haematopoietic stem cell transplantation across human leukocyte antigen barriers in dyskeratosis congenita. Bone Marrow Transplant 2003; 31: 847–850.

    Article  CAS  PubMed  Google Scholar 

  7. Ostranoff F, Ostranoff M, Calixto R, Florencio R, Domingues MC, Maior APS et al. Fludarabine, cyclophosphamide, and antithymocyte globulin for a patient with dyskeratosis congenita and severe bone marrow failure. Biol Blood Marrow Transplant 2007; 13: 366–368.

    Article  Google Scholar 

  8. Brazzola P, Duval M, Fournet JC, Gauvin F, Dalle JH, Champagne MA et al. Fatal diffuse capillaritis after haematopoietic stem cell transplantation for dyskeratosis congenita despite low-intensity conditioning regimen. Bone Marrow Transplant 2005; 36: 1103–1105.

    Article  CAS  PubMed  Google Scholar 

  9. Kottaridis PD, Milligan DW, Chopra R, Chakraverty RK, Chakrabarti S, Robinson S et al. In vivo Campath-1H prevents graft versus host disease following non-myeloablative stem cell transplantation. Blood 2000; 96: 2419–2425.

    CAS  PubMed  Google Scholar 

Download references

Author information

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
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. C Dalley
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. I Dokal
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A Laurie
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. V Gupta
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. J Marsh
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J Marsh.

Rights and permissions

Reprints and permissions

About this article

Cite this article

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

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.bmt.1705839

This article is cited by

Search

Advanced search

Quick links