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Vaiselbuh SR, Bryceson YT, Allen CE, Whitlock JA, Abla O |
Updates on histiocytic disorders. |
Pediatric blood & cancer 2014, 61: 1329 |
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Histiocytic disorders are rare entities that are becoming more recognized as our understanding of the molecular pathogenesis lead to novel diagnostic tests and targeted drug development. A symposium held at the American Society of Pediatric Hematology/Oncology (ASPHO) 2013 Annual Meeting discussed new insights into histiocytic disorders. This review highlights the symposium presentations, divided into three sections encompassing Langerhans cell histiocytosis (LCH), hemophagocytic lymphohistiocytosis (HLH) and Rosai Dorfman disease (RDD) including subsections on pathogenesis, clinical diagnostic criteria and novel insights into treatment. Details of other histiocytic disorders as well as the standard treatment guidelines have been published elsewhere and are beyond the scope of this discussion [Haupt et al. (2013). Pediatr Blood Cancer 60:175-184; Henter et al. (2007). Pediatr Blood Cancer 48:124-131]. Pediatr Blood Cancer 2014;61:1329-1335. © 2014 Wiley Periodicals, Inc. |
Valladeau J, Ravel O, Dezutter-Dambuyant C, Moore K, Kleijmeer M, Liu Y, Duvert-Frances V, Vincent C, Schmitt D, Davoust J, Caux C, Lebecque S, Saeland S |
Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules. |
Immunity 2000, 12: 71 |
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We have identified a type II Ca2+-dependent lectin displaying mannose-binding specificity, exclusively expressed by Langerhans cells (LC), and named Langerin. LC are uniquely characterized by Birbeck granules (BG), which are organelles consisting of superimposed and zippered membranes. Here, we have shown that Langerin is constitutively associated with BG and that antibody to Langerin is internalized into these structures. Remarkably, transfection of Langerin cDNA into fibroblasts created a compact network of membrane structures with typical features of BG. Langerin is thus a potent inducer of membrane superimposition and zippering leading to BG formation. Our data suggest that induction of BG is a consequence of the antigen-capture function of Langerin, allowing routing into these organelles and providing access to a nonclassical antigen-processing pathway. |
Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH |
Pulmonary Langerhans'-cell histiocytosis. |
The New England journal of medicine 2000 Jun 29; 342: 1969 |
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Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH |
Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. |
The New England journal of medicine 2002 Feb 14; 346: 484 |
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Pulmonary Langerhans'-cell histiocytosis is an uncommon interstitial lung disease in adults. It has an unpredictable course and may be associated with an increased susceptibility to the development of malignant neoplasms. |
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