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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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References

  1. Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH . Pulmonary Langerhans’-cell histiocytosis. N Engl J Med 2000; 342: 1969–1978.

    Article  CAS  Google Scholar 

  2. Minkov M, Grois N, Heitger A, Potschger U, Westermeier T, Gadner H . Response to initial treatment of multisystem Langerhans cell histiocytosis: an important prognostic indicator. Med Pediatr Oncol 2002; 39: 581–585.

    Article  Google Scholar 

  3. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ . Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer 1999; 85: 2278–2290.

    Article  CAS  Google Scholar 

  4. Akkari V, Donadieu J, Piguet C, Bordigoni P, Michel G, Blanche S et al. Hematopoietic stem cell transplantation in patients with severe Langerhans cell histiocytosis and hematological dysfunction: experience of the French Langerhans cell study group. Bone Marrow Transplant 2003; 31: 1097–1103.

    Article  CAS  Google Scholar 

  5. Kinugawa N, Imashuku S, Hirota Y, Yamada K, Yamamoto A, Akazai A et al. Hematopoietic stem cell transplantation (HSCT) for Langerhans cell histiocytosis (LCH) in Japan. Bone Marrow Transplant 1999; 24: 935–938.

    Article  CAS  Google Scholar 

  6. Steiner M, Matthes-Martin S, Attarbaschi A, Minkov M, Grois N, Unger F et al. Improved outcome of treatment-resistant high-risk Langerhans cell histiocytosis after allogeneic stem cell transplantation with reduced-intensity conditioning. Bone Marrow Transplant 2005; 36: 215–225.

    Article  CAS  Google Scholar 

  7. Jordan MB, McClain KL, Yan X, Hicks J, Jaffe R . Anti-CD52 antibody, alemtuzumab, binds to Langerhans cells in Langerhans cell histiocytosis. Pediatr Blood Cancer 2005; 44: 251–254.

    Article  Google Scholar 

  8. Ng CS, Desai SR, Rubens MB, Padley SP, Wells AU, Hansell DM . Visual quantitation and observer variation of signs of small airways disease at inspiratory and expiratory CT. J Thorac Imaging 1999; 14: 279–285.

    Article  CAS  Google Scholar 

  9. Vassallo RRJ, Colby T, Hartman T, Limper A . Pulmonary Langerhans’-cell histiocytosis. N Engl J Med 2005; 342: 1969–1978.

    Article  Google Scholar 

  10. Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH . Clinical outcomes of pulmonary Langerhans’-cell histiocytosis in adults. N Engl J Med 2002; 346: 484–490.

    Article  Google Scholar 

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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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  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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