慢性骨髄増殖性疾患における骨髄線維化の機序

抄録

For the purpose of elucidating the mechanism of myelofibrosis in chronic myeloproliferative disorders (CMD), collagen synthesis of bone marrow fibroblasts (BMF), collagen synthesis stimulated by platelets and collagenase activity were measured in the patients with CMD. The collagen synthesis of BMF from patients with chronic myelogenous leukemia (CML) and polycythemia vera (PV) was significantly greater than that from normal subjects. The platelets of patients with CML, PV and essential thrombocythemia stimulated BMF collagen synthesis significantly compared to the response from normal subjects. The collagenase activity of serum, peripheral leukocytes, bone marrow nucleated cells and BMF from CMD patients was observed to increase significantly greater than that from normals. Moreover, collagenase activity of CML patients with myelofibrosis was significantly higher than that of CML patients without myelofibrosis. From these results, it has been considered that myelofibrosis complicated with CMD occurs due to increased collagen synthesis by BMF and overproduction of collagen stimulated by platelets and resultantly collagenase activity increases.