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Autologous stem cell transplantation for refractory Langerhans' cell histiocytosis

Bone Marrow Transplantation volume 40, pages 807–808 (2007)Cite this article

Langerhans' cell histiocytosis (LCH) is a clinical entity characterized by proliferation of Langerhans' giant cells, and encompasses three distinct diseases: eosinophilic granuloma, Hand–Schüller–Christian disease, and Letterer–Siwe disease.1, 2 Eosinophilic granuloma primarily occurs in children and young adults, and is predominantly located in the skull.3 It usually develops as a solitary lesion, but may occur at multiple sites.3 Treatment of severe or chronic LCH that relies on traditional cytotoxic chemotherapy remains controversial and, in many cases, proves ineffective. Since LCH derives from hyperactivation and proliferation of macrophage progeny cells, there is a rationale for using BMT to treat advanced, resistant LCH.4 However, to our knowledge, there have been no reports of autologous peripheral blood stem cell transplantation (aPBSCT) for adult eosinophilic granuloma. We describe a patient in whom aPBSCT was performed for disseminated, refractory eosinophilic granuloma.

LCH is a rare disorder with variable clinical manifestations.2 A definitive diagnosis of LCH is made by histopathological evidence of Birbeck granules and immunohistochemical detection of S-100 and CD1a antigens in the tissue samples.2 In the present case, histological examination also revealed positive staining for CD1a and S-100 protein. LCH may sometimes result in life-threatening multisystem diseases. This depends on the involvement of ‘risk organ’ (that is, hematopoietic system, liver, spleen, and lungs).5 Thus, the present case was thought to be high-risk LCH, since she manifested abnormalities of the hematopoietic system and lungs. Various treatments have been employed for LCH, but not always with successful outcomes. Some authors recommend conservative treatment with biopsy and immobilization and the use of steroids.6 Although patients with localized disease may have an excellent outcome with minimal or no treatment, a significant proportion of patients with disseminated disease may undergo progression to a fatal outcome despite chemotherapy5 and immunotherapy.6 Since LCH derives from hyperactivation and proliferation of macrophage progeny cells, there is a rationale for the use of allogeneic BMT in treating advanced, high-risk or resistant LCH.7, 8 In particular, the successful allogeneic BMT performed by Ringden et al.8 casts new light on LCH patients failing other therapeutic approaches.5, 8, 9 In contrast, autologous marrow rescue has been considered a less attractive option due to the inherent tendency of the marrow to give rise to LCH. However, a case of effective autologous BMT for LCH is also reported, although it is a rare case.4 In addition, in most of the pediatric patients, myeloablative high-dose regimens are associated with a high treatment-related morbidity and mortality, and mobilizing stem cells from pediatric patients may be difficult.4, 9 In our patient, some risk factors for LCH resolved after aPBSCT and she maintained complete remission for 10 months, having never previously experienced such a stable period. Thus, aPBSCT performed for LCH can be considered as a second treatment choice for patients with no acceptable donor.

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Authors and Affiliations

  1. Division of Hematology, Kishiwada City Hospital, Kishiwada, Osaka, Japan

    K Ichikawa, S Nomura, K Ishii, M Okuno & C Kasai

  2. Division of Orthopedics, Kishiwada City Hospital, Kishiwada, Osaka, Japan

    T Maekawa

  3. Division of Pathology, Kishiwada City Hospital, Kishiwada, Osaka, Japan

    E Kadota

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  1. K Ichikawa
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  2. S Nomura
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Correspondence to S Nomura.

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Ichikawa, K., Nomura, S., Ishii, K. et al. Autologous stem cell transplantation for refractory Langerhans' cell histiocytosis. Bone Marrow Transplant 40, 807–808 (2007). https://doi.org/10.1038/sj.bmt.1705822

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