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Unrelated cord blood transplantation in an infant with severe multisystem Langerhans cell histiocytosis: clinical outcome, engraftment and culture of monocyte-derived dendritic cells

Bone Marrow Transplantation volume 33, pages 875–876 (2004)Cite this article

Langerhans cell histiocytosis (LCH) is a rare disease of still unknown etiology1 characterized by the accumulation of abnormal Langerhans cells in locations where they are normally not found. Langerhans cells belong to a family of antigen-presenting cells called dendritic cells.2 LCH cells resemble normal Langerhans cells with regard to the expression of S-100 neuroprotein and CD1a antigen. They contain the characteristic Birbeck granules. In addition to LCH cells, skin and organ infiltrates contain T lymphocytes, multinucleated giant histiocytes and eosinophils. Langerhans cells produce various cytokines (eg prostaglandin E2, IL-1, IL-3, IL-4, IL-8, GM-CSF and TNFα), which are thought to play a pivotal role in the pathogenesis of LCH.3 Patients may suffer from local disease primarily involving skin and bone or from multisystem disease, which is typically seen in children younger than 2 years. This results in a plethora of symptoms ranging from skin lesions and bone fractures to diabetes insipidus and bone marrow suppression, massive hepatosplenomegaly, lung infiltration and wasting. To improve therapeutic regimens in this rare disease, patients should be enrolled in international randomized clinical treatment studies, such as those sponsored by the Histiocyte Society (www.histio.org/society/).

We report umbilical cord blood transplantation from an unrelated donor with a single HLA-mismatch at the B locus and engraftment of donor hematopoiesis in a 15-month-old infant with severe multisystem LCH. Donor monocyte-derived dendritic cells (moDCs) could be cultured indicating engraftment of fully functional monocytes.

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  1. Department of Pediatrics, Children's Cancer Research Center/Biocenter, Martin-Luther-University Halle-Wittenberg, Halle, Germany

    F Meyer-Wentrup, J Foell, A Wawer & S Burdach

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  1. F Meyer-Wentrup
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  2. J Foell
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  3. A Wawer
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  4. S Burdach
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Meyer-Wentrup, F., Foell, J., Wawer, A. et al. Unrelated cord blood transplantation in an infant with severe multisystem Langerhans cell histiocytosis: clinical outcome, engraftment and culture of monocyte-derived dendritic cells. Bone Marrow Transplant 33, 875–876 (2004). https://doi.org/10.1038/sj.bmt.1704441

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