| 规范和重视原发性骨髓纤维化的诊断和治疗 |
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| 引用本文： | 肖志坚. 规范和重视原发性骨髓纤维化的诊断和治疗[J]. 白血病．淋巴瘤, 2010, 19(9): 513-516,522. DOI: 10.3760/cma.j.issn.1009-9921.2010.09.001 |
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| 作者姓名： | 肖志坚 |
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| 作者单位： | 中国医学科学院,北京协和医学院血液病医院,天津,300020 |
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| 基金项目： | 卫生行业科研专项经费项目 |
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| 摘 要： | 近五年相继提出了原发性骨髓纤维化（PMF）的骨髓纤维化程度判断欧洲共识标准、国际预后积分系统（IPSS）和疗效标准,为PMF的规范化诊断和治疗奠定了基础。免疫调节剂如沙利度胺、雷利度胺和pomalidomide单独或联合小剂量泼尼松临床试验的完成,使得除造血干细胞移植以外的药物治疗逆转骨髓纤维化程度成为可能,新近JAK2抑制剂临床试验的开展使PMF进入分子靶向治疗新时代。文章结合国际上PMF研究的新认识,重点探讨了中国在该领域应引起重视的几个问题。
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| 关 键 词： | 原发性骨髓纤维化 诊断 治疗 预后 |
| 收稿时间： | 2010-09-09; |
The state of diagnosis and treatment of patients with primary myelofibrosis |
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| XIAO Zhi-jian. The state of diagnosis and treatment of patients with primary myelofibrosis[J]. Journal of Leukemia & Lymphoma, 2010, 19(9): 513-516,522. DOI: 10.3760/cma.j.issn.1009-9921.2010.09.001 |
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| Authors: | XIAO Zhi-jian |
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| Affiliation: | XIAO ZAi-jian. (Institute of Hematology and Blood Diseases Hospital, CAMS and PUMC, Tianjin 300020, China) |
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| Abstract: | During the past few years, European consensus on grading bone marrow fibrosis, the Dynamic International Prognostic Scoring System (DIPSS) for primary myelofibrosis(PMF) and response criteria for PMF were developed, and some new drugs, such as the immunomodulator drugs (IMiDs) and JAK2 inhibitor are available for PMF. The recent advances were summarized and future issues were discussed. |
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| Keywords: | Primary myelofibrosis Diagnosis Treatment Prognosis |
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