F Fioredda, A Beccaria, P Casartelli, E Turrini, C Contratto, M C Giarratana, F Bagnasco, F Saettini, M Pillon, A Marzollo, S Zanardi, A Civino, D Onofrillo, M Lanciotti, I Ceccherini, A Grossi, D Coviello, P Terranova, M Lupia, G Del Borrello, P Uva, D Cangelosi, G Cavalca, M Miano, C Dufour
This study identifies a new chronic form of immune neutropenia in the young with or without detectable indirect anti-neutrophil antibodies, characterized by mild/moderate neutropenia low risk of severe infection (14%), tendency to develop autoimmune phenomena over the course of the disease (cumulative incidence of 58.6% after 20 years of disease duration), leukopenia, progressive reduction of absolute lymphocyte count and a T- and B-cell profile similar to autoimmune disorders like Sjogren syndrome, rheumatoid arthritis, and systemic lupus erythematosus (increased HLADR+ and CD3 + TCRγδ cells, reduced T regulatory cells, increased double-negative B and a tendency to reduced B memory cells)...
January 29, 2024: American Journal of Hematology