原发性骨髓纤维化脾肿大发生机制及靶向治疗进展  

Pathogenesis and Targeted Treatment Progress of Splenomegaly in Primary Myelofibrosis——Review

作　　者：陈紫薇 王诗轩[1] 李菲 CHEN Zi-Wei;WANG Shi-Xuan;LI Fei(Center of Hematology,The First Affiliated Hospital of Nanchang University,Nanchang 330006,Jiangxi Province,China;Institute of Hematology,Academy of Clinical Medicine of Jiangxi Province,Nanchang 330006,Jiangxi Province,China;Institute of Lymphoma of Nanchang University,Nanchang 330006,Jiangxi Province,China)

机构地区：[1]南昌大学第一附属医院血液科,江西南昌330006 [2]江西省临床医学科学研究院血液病研究所,江西南昌330006 [3]南昌大学淋巴肿瘤疾病研究所,江西南昌330006

出　　处：《中国实验血液学杂志》2024年第1期308-312,共5页Journal of Experimental Hematology

基　　金：国家自然科学基金(82060030);江西省科技创新基地计划-江西省血液病临床医学研究中心(20212BCG74001,20211ZDG02006)。

摘　　要：原发性骨髓纤维化是一种骨髓增殖性肿瘤,脾肿大是其突出的临床特征,通常被认为与脾脏的髓外造血有关。CXCL12/CXCR4轴的异常可以导致造血干细胞和造血祖细胞从骨髓迁移到脾脏从而引起脾内髓外造血。此外,低GATA1表达和细胞因子异常分泌被发现与脾肿大显著相关。随着JAK1/2抑制剂在临床中的使用,原发性骨髓纤维化患者的脾大症状得到了显著改善。本文将对原发性骨髓纤维化脾肿大的发生机制及靶向治疗研究进展作一综述。Primary myelofibrosis(PMF)is a myeloproliferative neoplasm with splenomegaly as the major clinical manifestation,which is commonly considered to be linked to splenic extramedullary hematopoiesis.Alteration of CXCL12/CXCR4 pathway can lead to the migration of hematopoietic stem cells and hematopoietic progenitor cells from bone marrow to spleen which results in splenic extramedullary hematopoiesis.In addition,low GATA1 expression and the abnormal secretion of cytokines were found to be significantly associated with splenomegaly.With the application of JAK1/2 inhibitors in clinical,the symptoms of splenomegaly have been significantly improved in PMF patients.This article will review the pathogenesis and targeted treatment progress of splenomegaly in PMF.

关 键 词：原发性骨髓纤维化 脾肿大 髓外造血 靶向治疗 

分 类 号：R733.3[医药卫生—肿瘤]