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• W178485222 endingPage "3528" @default.
• W178485222 startingPage "3522" @default.
• W178485222 abstract "Peripheral blood B cells in patients with paroxysmal nocturnal hemoglobinuria (PNH) comprise variable mixtures of normal B cells produced before the onset of disease and glycosylphosphatidylinositol (GPI)-deficient B cells derived from the PNH hematopoietic stem cell. In a detailed phenotypic analysis of 29 patients with PNH, this study shows consistent phenotypic differences between PNH B cells and residual normal B cells. In the majority of patients with active disease, PNH B cells comprised mainly naive cells with a CD27−IgM+IgDstrong+IgG−phenotype. The proportion of CD27+ memory cells within this compartment was related to disease duration (Spearman [rs] 0.403; P = .030). In PNH patients with predominantly GPI-deficient hematopoiesis, that is, a large granulocyte PNH clone, the residual normal B cells had a predominantly memory (CD27+) phenotype. Furthermore, the majority of these memory B cells were not immunoglobulin (Ig) class switched and had an IgM+IgD+IgG− phenotype. Using PNH as a novel model with which to study B lymphopoiesis, this study provides direct evidence that production of new naive B cells occurs throughout life and that the major population of long-lived memory B cells are IgM+IgD+. Moreover, studies of GPI− B cells in 2 patients in remission from PNH suggest that the life span of a B-cell clone can be more than 24 years." @default.
• W178485222 created "2016-06-24" @default.
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• W178485222 date "2000-11-15" @default.
• W178485222 modified "2023-09-28" @default.
• W178485222 title "Immunophenotypic analysis of B cells in PNH: insights into the generation of circulating naive and memory B cells" @default.
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• W178485222 doi "https://doi.org/10.1182/blood.v96.10.3522.h8003522_3522_3528" @default.
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