Abstract
Primary autoimmune neutropenia (pAN) is typified by onset in early infancy and a mild/moderate phenotype that resolves within 3 years of diagnosis. In contrast, secondary AN is classically an adult disease associated with malignancy, autoimmunity, immunodeficiency, viral infection, or drugs. This study describes a cohort of 79 children from the Italian Registry who, although resembling pAN, did not fully match the criteria for pAN because neutropenia either appeared after age 5 years (LO-Np) or lasted longer than 3 years (LL-Np). These 2 categories compared with classical pAN showed a far inferior rate of resolution (P < .001), lower severity of neutropenia (P = .03), leukopenia (P < .001), lymphopenia (P < .001) with low B+ (P = .001), increased need of granulocyte colony-stimulating factor (P = .04), and increased frequency of autoimmunity over the disease course (P < .001). A paired comparison between LO-Np and LL-Np suggested that LO-Np had a lower rate of resolution (P < .001) and lower white blood cell (P < .001) and lymphocyte (P < .001) values, higher occurrence of apthae (P = .008), and a stronger association with autoimmune diseases/markers (P = .001) than LL-Np, thus suggesting a more pronounced autoimmune signature ...Continue Reading
References
Jan 25, 2005·Vox Sanguinis·M BruinM de Haas
Jul 12, 2005·Nature Genetics·U SalzerB Grimbacher
Oct 7, 2005·Arthritis Research & Therapy·Franco CapsoniAlberto Zanella
Nov 7, 2007·The Journal of Allergy and Clinical Immunology·Li ZhangCharlotte Cunningham-Rundles
Aug 2, 2008·Blood·Amanda J WalneInderjeet Dokal
Apr 28, 2009·Blood·Douglas B CinesEline T Luning Prak
Mar 25, 2010·Blood·Charlotte Cunningham-Rundles
Jan 4, 2012·American Journal of Hematology·Francesca FioreddaUNKNOWN Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato-Oncologia Pediatrica)
May 15, 2013·Vox Sanguinis·G LucasJ Bux
Sep 12, 2015·American Journal of Hematology·Piero FarruggiaCarlo Dufour
Jan 16, 2016·The Journal of Allergy and Clinical Immunology·Laura Gámez-DíazBodo Grimbacher
Jun 2, 2017·American Journal of Hematology·Piero FarruggiaCarlo Dufour
Feb 19, 2019·The Journal of Allergy and Clinical Immunology. in Practice·Markus G SeidelUNKNOWN ESID Registry Working Party and collaborators
Oct 9, 2019·Expert Review of Clinical Immunology·Mohammad GhorbaniAsghar Aghamohammadi