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原发性骨髓纤维化系一种原因不明的以纤维母细胞和单母细胞为主的骨髓增殖性疾病。其他如真性红血球增多症、原发性血小板增多症、慢性髓性白血病等到晚期也可发展为骨髓纤维化,但均可伴有代偿性髓外造血系统如肝、脾和淋巴结等的增生。根据文献记载,本病有X线表现的约为40％。本文就23例经临床和骨穿病理证实的原发性骨髓纤维化中有明显骨骼X线改变的7例作一分析。
Primary myelofibrosis is an unexplained myeloproliferative disorder characterized by fibroblasts and single blast cells. Others such as polycythemia vera, essential thrombocythemia, chronic myeloid leukemia can also be advanced to fibrosis, but may be associated with compensatory extramedullary hematopoietic systems such as liver, spleen and lymph node proliferation. According to the literature, this disease has about 40% X-ray findings. In this paper, 23 cases of primary bone marrow fibrosis confirmed by clinical and bone biopsy showed significant skeletal X-ray changes in an analysis of 7 cases.