Myelofibrosis and the biology of connective tissue
Myelofibrosis and the biology of connective tissue
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骨髓纤维化和结缔组织生物学
DOI：
10.1038/bjc.1985.214
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发表时间：
1985
影响因子：
8.8
通讯作者：
G. Geary
中科院分区：
文献类型：
--
作者：
G. Geary
在过去的十年中，我们对慢性骨髓增殖性疾病的病理生理学的理解取得了重要进展，但经常使这种综合征复杂化的骨髓纤维化现象仍然是一个谜。本书记录了在西奈山医学院举办的骨髓纤维化研讨会的会议记录。在介绍了骨髓纤维化的临床方面和自然史的两章之后，本书的其余部分包括对结缔组织生物学的贡献，以及肿瘤性造血刺激骨髓基质细胞导致网状蛋白或胶原蛋白在骨髓中沉积的方式。讨论的许多主题包括胶原蛋白合成及其降解的调节，巨核细胞、血小板和其他血细胞产生的生长因子对结缔组织细胞的有丝分裂刺激；正常个体和骨髓纤维化患者骨髓成纤维细胞的培养行为和特性；以及其他慢性疾病（如肺纤维化和硬皮病）以及特发性骨髓纤维化本身的纤维化发病机制。这本书的最后一个有趣的章节介绍了使用秋水仙碱或青霉胺等药物溶解骨髓纤维化的新方法。然而，可能有人会说，如果骨髓纤维化是造血障碍的一种副现象，那么在不治疗潜在疾病的情况下单独治疗它可能不会有利可图。本书将特别吸引实验血液学家和细胞生物学家，但任何与患有这种顽固性疾病的患者打交道的临床医生都会对它感兴趣，迄今为止，这种材料很少出现在血液学教科书中。
During the last decade, there have been important developments inour understanding of the pathophysiology of the chronic myeloproliferative disorders, but the phenomenon of myelofibrosis, which often complicates this syndrome, remains something of an enigma. This book represents the proceedings of a Symposium on myelofibrosis held at the Mount Sinai School of Medicine. After two introductory chapters on the clinical aspects and natural history of myelofibrosis, the rest of the book consists of contributions on the biology of connective tissue, and the way in which stimulation of bone marrow stromal cells by neoplastic haemopoiesis results in deposition of reticulin or collagen in the marrow. The many subjects discussed include the regulation of collagen synthesis and its degradation, the mitogenic stimulation of connective tissue cells by growth factors produced by megakaryocytes, platelets and other blood cells; the cultural behaviour and properties of bone marrow fibroblast in normal individuals and in patients with myelofibrosis; and the pathogenesis of fibrosis in other chronic disorders such as pulmonary fibrosis and scleroderma, as well as in idiopathic myelo-fibrosis itself. The book ends with an interesting chapter on new approaches to the therapeutic dissolution of bone marrow fibrosis using agents such as Colchicine or Penicillamine. It might be argued, though, that if myelofibrosis is an epiphenomenon of disordered haemopoiesis, it, may not be profitable to treat this alone, without therapy for the underlying disorder. This book will appeal particularly to the experimental haematologist and cell biologist, but ttny clinician dealing with patients suffering from this intractable and inexorable disease will find it of interest, very little of this material having so far appeared in haematological textbooks.