早期/纤维化前原发性骨髓纤维化的研究进展

国际输血及血液学杂志 Pub Date : 2018-11-20 DOI:10.3760/CMA.J.ISSN.1673-419X.2018.06.009

Zhiyang Liu, Xu-liang Shen

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引用次数: 0

摘要

早期/纤维化前原发性骨髓纤维化（pre-PMF）是原发性脊髓纤维化（PMF）的早期阶段。它是一种骨髓增生性肿瘤，临床表现迟钝，预后良好。PMF前期的诊断主要取决于骨髓形态和次要临床标准。PMF前期患者的预后比PMF患者好。本文就PMF前期的骨髓形态、临床和实验室特征、基因突变和负荷、治疗和预后等方面的研究进展进行综述，以提高对该疾病的认识和诊断。关键词：原发性骨髓纤维化；巨核细胞；基因表达调控；药物治疗；造血干细胞移植；预后

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Research progress of early/prefibrotic primary myelofibrosis

Early/prefibrotic primary myelofibrosis (pre-PMF) is an early stage of primary myelofibrosis (PMF). It is a myeloproliferative neoplasms (MPN) with inert clinical manifestations and good prognosis. Diagnosis of pre-PMF mainly depends on bone marrow morphology and secondary clinical criteria. Patients with Pre-PMF have a better prognosis than those of PMF patients. This article reviews the research progress of bone marrow morphology, clinical and laboratory characteristics, gene mutation and load, treatment and prognosis of pre-PMF in order to improve understanding and diagnosis of the disease. Key words: Primary myelofibrosis; Megakaryocytes; Gene expression regulation; Drug therapy; Hematopoietic stem cell transplantation; Prognosis

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来源期刊

国际输血及血液学杂志

自引率

0.00%

发文量

10610

期刊介绍： The International Journal of Transfusion and Hematology was founded in September 1978. It is a comprehensive academic journal in the field of transfusion and hematology, supervised by the National Health Commission and co-sponsored by the Chinese Medical Association, West China Second Hospital of Sichuan University, and the Institute of Transfusion Medicine of the Chinese Academy of Medical Sciences. The journal is a comprehensive academic journal that combines the basic and clinical aspects of transfusion and hematology and is publicly distributed at home and abroad. The International Journal of Transfusion and Hematology mainly reports on the basic and clinical scientific research results and progress in the field of transfusion and hematology, new experiences, new methods, and new technologies in clinical diagnosis and treatment, introduces domestic and foreign research trends, conducts academic exchanges, and promotes the development of basic and clinical research in the field of transfusion and hematology.