Cloning evolution mechanism of paroxysmal nocturnal hemoglobinuria
ZHOU Rui-qing
WANG Shun-qing
Abstract：The core pathogenesis of paroxysmal nocturnal hemoglobinuria(PNH)is that somatic mutation of PIG-A gene on X chromosome leads to the deletion of glycosyl phosphatidylinositol anchor proteins such as CD55 and CD59,and the vulnerability of red blood cells to complement attack increases,resulting in hemolysis.The dynamic evolution of PNH clone is closely related to immune microenvironment pressure,secondary mutation and treatment intervention,and often interacts with bone marrow failure diseases and myeloid tumors.This paper reviews the pathogenesis,clonal evolution model and clinical significance of PNH,and discusses the role of immune microenvironment,gene mutation and treatment choice in disease progression,so as to provide theoretical basis for optimizing diagnosis and treatment.
Keywords：paroxysmal nocturnal hemoglobinuriaPIG-A geneclonal evolutionimmune escapebone marrow failuremyelodysplastic syndrome
Publication Date：2025-07-01
Online Publishing Date：2025-09-04(First online date of this platform, not the publication date of the document)
Pages：6( 570-575 )