Smith B D, Moliterno A R
Johns Hopkins University School of Medicine, Baltimore, Maryland 21231, USA.
Curr Opin Oncol. 2001 Mar;13(2):91-4. doi: 10.1097/00001622-200103000-00002.
Idiopathic myelofibrosis (IMF) is a hematopoietic stem cell disorder characterized by hypercellularity of the bone marrow with an increase in abnormal megakaryocytes, varying degrees of marrow fibrosis, and extramedullary hematopoiesis. The central lesion of the IMF stem cell is not known; however, the marrow fibrosis is a polyclonal reaction to inflammatory mediators generated by the transformed clone. Historical management approaches have centered on improving the patient's blood counts in a palliative manner. Recent reports of autologous and allogeneic bone marrow transplantation (BMT) in IMF patients indicate that stable engraftment occurs easily, the marrow fibrosis is a reversible process, and a graft-versus-fibrosis effect may exist. The goals of future therapies will target marrow fibrosis and harness the graft-versus-fibrosis effect to safely and effectively treat patients with IMF.
原发性骨髓纤维化（IMF）是一种造血干细胞疾病，其特征为骨髓细胞增多，异常巨核细胞增加，伴有不同程度的骨髓纤维化和髓外造血。目前尚不清楚IMF干细胞的核心病变；然而，骨髓纤维化是对转化克隆产生的炎症介质的多克隆反应。以往的治疗方法主要是以姑息方式改善患者的血细胞计数。最近关于IMF患者自体和异基因骨髓移植（BMT）的报告表明，移植容易稳定植入，骨髓纤维化是一个可逆过程，可能存在移植物抗纤维化效应。未来治疗的目标将针对骨髓纤维化，并利用移植物抗纤维化效应来安全有效地治疗IMF患者。
