慢性骨髄増殖性疾患におけるPDGF活性

抄録

Platelet derived growth factor (PDGF) has been suggested to play some role in the pathogenesis of myelofibrosis frequently encountered in patients with myeloproliferative disorders (MPD). In this study we measured PDGF activity in circulating platelets of patients with MPD, and investigated the growth of marrow fibroblasts from MPD patients using PDGF in the presence of human plasma derived serum.
PDGF activity of MPD patients was lower than that of normal controls, and the activity in patients with myelofibrosis was slightly lower than in those without fibrosis, supporting the idea that an abnormal release of PDGF from megakaryocytes in the bone marrow environment induce a marrow fibrosis. The PDGF-stimulated proliferation of marrow fibroblasts in MPD patients was increased compared to normal individuals. This increment of proliferation might be due to higher sensitivity of the fibroblasts to plasma derived serum than to PDGF, indicating that MPD fibroblasts are more sensitive to some factor(s) in plasma. These results could explain the pathogenesis of myelofibrosis in MPD patients.