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Blood, 15 October 2008, Vol. 112, No. 8, pp. 3026-3035. Prepublished online as a Blood First Edition Paper on July 31, 2008; DOI 10.1182/blood-2008-06-158386. This Article Full Text (PDF) Supplemental Appendix All Versions of this Article: blood-2008-06-158386v1 112/8/3026    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Lataillade, J.-J. Articles by Le Bousse-Kerdiles, M.-C. Search for Related Content PubMed PubMed Citation Articles by Lataillade, J.-J. Articles by Le Bousse-Kerdiles, M.-C. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted June 11, 2008 Accepted July 21, 2008 Does primary myelofibrosis involve a defective stem cell niche? From concept to evidence Jean-Jacques Lataillade, Olivier Pierre-Louis, Hans Carl Hasselbalch, Georges Uzan, Claude Jasmin, Marie-Claire Martyre, and Marie-Caroline Le Bousse-Kerdiles* Laboratoire de Recherche, CTSA-Hopital Percy, Clamart, France Inserm U602, Hopital Paul Brousse, Villejuif, France Department of Hematology, Herlev University Hospital, Copenhagen, Denmark Université Paris 11, IFR 89, Institut Andre Lwoff, Villejuif, France * Corresponding author; email: caroline.le-bousse-kerdiles{at}inserm.fr. Primary Myelofibrosis (PMF) is the rarest and the most severe Philadelphia negative chronic myeloproliferative syndrome. By associating a clonal proliferation and a mobilization of hematopoietic stem cells from bone marrow to spleen with profound alterations of the stroma, PMF is a remarkable model in which deregulation of the stem cell niche is of utmost importance for the disease development. This manuscript reviews key data suggesting that an imbalance between endosteal and vascular niches participates in the development of the clonal stem cell proliferation. Mechanisms by which bone marrow niches are altered with ensuing mobilization and homing of neoplastic hematopoietic stem cells in new or reinitialized niches in the spleen and liver are examined. Differences between signals delivered by both endosteal and vascular niches in the bone marrow and spleen of patients as well as the responsiveness of PMF stem cells to their specific signals are discussed. A proposal for integrating a potential role for the JAK2 mutation in their altered sensitivity is made. A better understanding of the crosstalk between stem cells and their niche should imply new therapeutic strategies targeting not only intrinsic defects in stem cell signalling but also regulatory hematopoietic niche-derived signals and, consequently, stem cell proliferation. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. W. Lane, D. T. Scadden, and D. G. Gilliland The leukemic stem cell niche: current concepts and therapeutic opportunities Blood, August 6, 2009; 114(6): 1150 - 1157. [Abstract] [Full Text] [PDF]

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