Transcriptome analysis of bone marrow mesenchymal stromal cells from patients with primary myelofibrosis.
Primary myelofibrosis (PMF) is a clonal myeloproliferative neoplasm whose severity and treatment complexity are attributed to the presence of bone marrow (BM) fibrosis and alterations of stroma impairing the production of normal blood cells. Despite the recently discovered mutations including the JAK2V617F mutation in about half of patients, the primitive event responsible for the clonal proliferation is still unknown. In the highly inflammatory context of PMF, the presence of fibrosis associated with a neoangiogenesis and an osteosclerosis concomitant to the myeloproliferation and to the increase number of circulating hematopoietic progenitors suggests that the crosstalk between hematopoietic and stromal cells is deregulated in the PMF BM microenvironmental niches. Within these niches, mesenchymal stromal cells (BM-MSC) play a hematopoietic supportive role in the production of growth factors and extracellular matrix which regulate the proliferation, differentiation, adhesion and migration of hematopoietic stem/progenitor cells. A transcriptome analysis of BM-MSC in PMF patients will help to characterize their molecular alterations and to understand their involvement in the hematopoietic stem/progenitor cell deregulation that features PMF.
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Christophe
C
Martinaud
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Christophe
C
Desterke
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Johanna
J
Konopacki
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Alessandro M
AM
Vannucchi
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Lisa
L
Pieri
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Paola
P
Guglielmelli
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Brigitte
B
Dupriez
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Jean-Christophe
JC
Ianotto
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Laetitia
L
Boutin
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Jean-Jacques
JJ
Lataillade
INSERM Unit 1197 and UMS33, Paul Brousse Hospital, Andre Lwoff Institute, France.
Marie-Caroline
MC
Le Bousse-Kerdilès
Bone marrow; Mesenchymal stroma cells; Myeloproliferative disorders; Primary myelofibrosis