NDT Advance Access originally published online on September 21, 2007
Nephrology Dialysis Transplantation 2007 22(12):3664-3667; doi:10.1093/ndt/gfm299
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
(Section Editor: A. Meyrier)
Histiocytosis X and renal insufficiency
1Department of Internal Medicine III, University of Jena and 2Department of Cellular and Molecular Pathology, German Cancer Research Center, Heidelberg, Germany
Correspondence and offprint requests to: Gunter Wolf, MD, Klinik für Innere Medizin III, University Hospital Jena, Erlanger Allee 101, D-07747 Jena, Germany. Email: gunter.wolf@med.uni-jena.de
Keywords: CD1a-positive cells; interstitial nephritis; Langerhans cell histiocytosis
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Langerhans cell histiocytosis (LCH) is a rare disorder showing a wide spectrum of clinical manifestations ranging from single cutaneous lesions to multifocal systemic disease [1,2]. The typical infiltration consists of pathological monoclonal Langerhans cells together with lymphocytes, eosinophilic granulocytes and non-dendritic histiocytes [3]. Langerhans cells are antigen-presenting cells with features of dendritic cells, staining positive for CD1a [4]. One of their original functions is cutaneous immunosurveillance. LCH is rare and the incidence in adults is 1–2 cases per million, with a mean age of 35 ± 14 years, and is slightly more prevalent in males [5,6]. Histological diagnosis is made if light microscopic morphological characteristics of the disease are found with positive staining for CD1a antigen [4,7]. In addition, Birbeck granules in the lesional cell that are detected with electron microscopy are also suggestive
Case
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