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Reduced-intensity conditioned allogeneic haematopoietic transplantation in an adult with Langerhans’ cell histiocytosis and thrombocytopenia with absent radii

Bone Marrow Transplantation volume 37, pages 713–715 (2006)Cite this article

Langerhans cell histiocytosis (LCH) is a rare disorder in adults, presenting as single organ or multisystem disease, with pulmonary disease occurring more frequently in isolation.1 Failure of chemotherapy is reported in 20% of cases with multisystem LCH with overall survival of only 20%.2, 3 Reports of myeloablative allogeneic stem cell transplantation in isolated cases of refractory LCH in children are described.4, 5 A high transplant-related mortality, particularly in the presence of pre-existing end-organ damage, limits its use.

A recent report of nine children with poor-risk LCH treated with reduced-intensity conditioned allogeneic haematopoietic stem cell transplantation (RIC allo-HSCT) showed encouraging results with 7/9 cases having no disease progression and alive at a median follow-up of 390 days with only one case showing non-engraftment.6 The benefit of alemtuzumab (Campath-1H) incorporated into the pre-transplant conditioning is twofold. A significant reduction in the incidence of GvHD is well described and in addition, Jordan et al.7 report the binding of alemtuzumab to pathological Langerhans cells (LCs) in LCH but not to normal LCs, suggesting a further target for therapy.

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Authors and Affiliations

  1. Department of Haematological Medicine, Kings College Hospital, London, UK

    W Ingram & G Mufti

  2. Department of Radiology, Kings College Hospital, London, UK

    S R Desai

  3. National Pulmonary Hypertension Service, Hammersmith Hospital, London, UK

    J S R Gibbs

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  1. W Ingram
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  2. S R Desai
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  3. J S R Gibbs
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  4. G Mufti
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Correspondence to G Mufti.

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Ingram, W., Desai, S., Gibbs, J. et al. Reduced-intensity conditioned allogeneic haematopoietic transplantation in an adult with Langerhans’ cell histiocytosis and thrombocytopenia with absent radii. Bone Marrow Transplant 37, 713–715 (2006). https://doi.org/10.1038/sj.bmt.1705300

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