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首页> 外文期刊>Current Immunology Reviews >Molecular Advances Toward the Understanding of the Patho-Biology of Idiopathic Myelofibrosis
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Molecular Advances Toward the Understanding of the Patho-Biology of Idiopathic Myelofibrosis

机译：对特发性骨髓纤维化病理生物学认识的分子进展

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摘要

Chronic idiopathic myelofibrosis (IM) is a chronic myeloproliferative disorder characterized by splenomegaly, a leukoerythroblastic blood picture, teardrop poikilocytosis, marrow fibrosis, osteosclerosis, marrow neo-vascularization, abnormal stem/progenitor cell trafficking and extramedullary hematopoiesis. The disease may eventually evolve into acute leukemia. This Philadelphia chromosome negative disorder is thought to originate from a somatic mutation at the level of the multipotent hematopoietic stem cell, the most visible consequence of which is a profound hyperplasia associated with increased proliferation but abnormal differentiation of the megakaryocytes (MKs). The pathobiology of the disease, however, involves not only abnormal hematopoietic stem/progenitor cells functions, but also a defective marrow microenvironment. The molecular nature of the genetic defect in IM and how this defect might induce so many pleiotropic consequences remains unknown. Many of the features of the human disease can be reproduced in mice by genetic alterations that induce MK abnormalities similar to those found in patients. Unfortunately, none of the mutations causing the disease in mice has been detected in the human disease. These animal models, however, allow one to dissect the patho-biological pathway that establishes the complex features of IM. Furthermore, these models also shed light on the cross-talk between stem/progenitor cells and microenvironment in normal hematopoiesis.
机译：慢性特发性骨髓纤维化（IM）是一种慢性骨髓增生性疾病，其特征是脾肿大，白细胞红血球图像，泪液性单核细胞增多症，骨髓纤维化，骨硬化，骨髓新血管形成，干/祖细胞运输异常和髓外造血。该疾病最终可能演变成急性白血病。人们认为这种费城染色体阴性疾病是由多能造血干细胞水平的体细胞突变引起的，其最明显的后果是与增生但巨核细胞（MKs）异常分化有关的深层增生。然而，该疾病的病理生物学不仅涉及造血干/祖细胞功能异常，而且还涉及有缺陷的骨髓微环境。 IM中遗传缺陷的分子性质以及该缺陷如何引发如此多效性的后果仍然未知。人类疾病的许多特征可以通过诱发MK异常的遗传改变在小鼠中复制，这种遗传改变与患者发现的异常相似。不幸的是，在人类疾病中没有发现引起小鼠疾病的突变。然而，这些动物模型允许解剖建立IM复杂特征的病理生物学途径。此外，这些模型还揭示了正常造血过程中干细胞/祖细胞与微环境之间的相互影响。

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