NDLI: In vivo transformation of mouse conventional CD8α+ dendritic cells leads to progressive multisystem histiocytosis

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In vivo transformation of mouse conventional CD8α+ dendritic cells leads to progressive multisystem histiocytosis

Content Provider	Scilit
Author	Steiner, Quynh-Giao Otten, Luc A. Hicks, M. John Kaya, Gürkan Grosjean, Frederic Saeuberli, Estelle Lavanchy, Christine Beermann, Friedrich McClain, Kenneth L. Acha-Orbea, Hans
Copyright Year	2008
Description	Division and proliferation of dendritic cells (DCs) have been proposed to contribute to homeostasis and to prolonged antigen presentation. Whether abnormal proliferation of dendritic cells causes Langerhans cell histiocytosis (LCH) is a highly debated topic. Transgenic expression of simian virus 40 (SV40) T antigens in mature DCs allowed their transformation in vivo while maintaining their phenotype, function, and maturation capacity. The transformed cells were differentiated splenic CD8 alpha–positive conventional dendritic cells with increased Langerin expression. Their selective transformation was correlated with higher steady-state cycling compared with CD8 alpha–negative DCs in wild-type and transgenic mice. Mice developed a DC disease involving the spleen, liver, bone marrow, thymus, and mesenteric lymph node. Surprisingly, lesions displayed key immunohistologic features of Langerhans cell histiocytosis, including expression of Langerin and absence of the abnormal mitoses observed in Langerhans cell sarcomas. Our results demonstrate that a transgenic mouse model with striking similarities to aggressive forms of multisystem histiocytosis, such as the Letterer-Siwe syndrome, can be obtained by transformation of conventional DCs. These findings suggest that conventional DCs may cause some human multisystem LCH. They can reveal shared molecular pathways for human histiocytosis between humans and mice.
Related Links	http://bloodjournal.hematologylibrary.org/content/111/4/2073.full.pdf
Ending Page	2082
Page Count	10
Starting Page	2073
DOI	10.1182/blood-2007-06-097576
Journal	Blood
Issue Number	4
Volume Number	111
Language	English
Publisher	American Society of Hematology
Publisher Date	2008-02-15
Access Restriction	Open
Subject Keyword	Research and Experimental Medicine Pathology Langerhans Cell Histiocytosis Bone Marrow Antigen Abnormal Dcs Vivo Transformation Multisystem Histiocytosis Journal: Blood (Vol- 78, Issue- 4)
Content Type	Text
Resource Type	Article

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