Pathogenesis and Targeted Treatment Progress of Splenomegaly in Primary Myelofibrosis

VernacularTitle:原发性骨髓纤维化脾肿大发生机制及靶向治疗进展
Author: Zi-Wei CHEN ¹ ; Shi-Xuan WANG ; Fei LI
Author Information

1. 南昌大学第一附属医院血液科;江西省临床医学科学研究院血液病研究所;南昌大学淋巴肿瘤疾病研究所,江西南昌 330006
Keywords: primary myelofibrosis; splenomegaly; extramedullary hematopoiesis; targeted treatment
From: Journal of Experimental Hematology 2024;32(1):308-312
CountryChina
Language:Chinese
Abstract: 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.