Late‐onset and long‐lasting neutropenias in the young: A new entity anticipating immune‐dysregulation disorders

• Published in: American journal of hematology Vol. 99; no. 4; pp. 534 - 542
• Main Authors: Fioredda, F., Beccaria, A., Casartelli, P., Turrini, E., Contratto, C., Giarratana, M. C., Bagnasco, F., Saettini, F., Pillon, M., Marzollo, A., Zanardi, S., Civino, A., Onofrillo, D., Lanciotti, M., Ceccherini, I., Grossi, A., Coviello, D., Terranova, P., Lupia, M., Del Borrello, G., Uva, P., Cangelosi, D., Cavalca, G., Miano, M., Dufour, C.
• Format: Journal Article
• Language: English
• Published: Hoboken, USA John Wiley & Sons, Inc 01.04.2024; Wiley Subscription Services, Inc
• Subjects: Autoimmune diseases; CD3 antigen; Cell number; Genetic counseling; Immunoregulation; Leukocytes (neutrophilic); Leukopenia; Lymphocytes; Lymphocytes T; Memory cells; Neutropenia; Rheumatoid arthritis; Sjogren's syndrome; Systemic lupus erythematosus
• ISSN: 0361-8609; 1096-8652; 1096-8652
• DOI: 10.1002/ajh.27221

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). In a minority of patients, P/LP variants related to primary immuno‐regulatory disorders were found. This new form may fit the group of “Likely acquired neutropenia,” a provisional category included in the recent International Guidelines on Diagnosis and Management of Neutropenia of EHA and EUNET INNOCHRON ACTION 18233. The early recognition of this form of neutropenia would help clinicians to delineate better specific monitoring plans, genetic counseling, and potentially targeted therapies. Main features of Autoimmune and Idiopathic Late Onset and Long Lasting Neutropenias.