Naslov
Does primary myelofibrosis involve a defective stem cell niche? From concept to evidence

Autori
Lataillade, J. J. ; Pierre-Louis, O. ; Hasselbalch, H. S. ; Uzan, G. ; Jasmin, C. ; Martyre, M. C. ; Le Bousse-Kerdiles, M. C. ; Kušec, Rajko ; French INSERM ; European EUMNET

Izvornik
Blood (0006-4971) 112 (2008), 8; 3026-3035

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, pregledni rad, znanstveni

Ključne riječi
primary myelofibrosis; stem cell; cell niche

Sažetak
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 paper reviews key data suggesting that an imbalance between endosteal and vascular niches participates in the development of 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 cross talk between stem cells and their niche should imply new therapeutic strategies targeting not only intrinsic defects in stem cell signaling but also regulatory hematopoietic niche-derived signals and, consequently, stem cell proliferation.

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti

Napomena
Koautori rada u prosirenoj autorskoj grupi su clanovi Europske mreze za istrazivanje mijelofibroze (EUMNET) ciji je clan te koautor rada i nosioc projekta - Rajko Kusec

POVEZANOST RADA