Activation of non-canonical TGF-β1 signaling indicates an autoimmune mechanism for bone marrow fibrosis in primary myelofibrosis
F Ciaffoni, E Cassella, L Varricchio, M Massa… - Blood Cells, Molecules …, 2015 - Elsevier
Primary myelofibrosis (PMF) is characterized by megakaryocyte hyperplasia, dysplasia and
death with progressive reticulin/collagen fibrosis in marrow and hematopoiesis in
extramedullary sites. The mechanism of fibrosis was investigated by comparing TGF-β1
signaling of marrow and spleen of patients with PMF and of non-diseased individuals.
Expression of 39 (23 up-regulated and 16 down-regulated) and 38 (8 up-regulated and 30
down-regulated) TGF-β1 signaling genes was altered in the marrow and spleen of PMF …
death with progressive reticulin/collagen fibrosis in marrow and hematopoiesis in
extramedullary sites. The mechanism of fibrosis was investigated by comparing TGF-β1
signaling of marrow and spleen of patients with PMF and of non-diseased individuals.
Expression of 39 (23 up-regulated and 16 down-regulated) and 38 (8 up-regulated and 30
down-regulated) TGF-β1 signaling genes was altered in the marrow and spleen of PMF …