Beccaria, Andrea

Dufour, Carlo

Fioredda, Francesca

English

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67 Adresa za dopisivanje:Carlo Dufour, Hematology Unit. Department of Hemato Oncology – IRCCS G. Gaslini Children’s Hospital, Genova, ItalyLiječ Vjesn 2023;145;suplement 5:67 https://doi.org/10.26800/LV-145-supl5-15Long Lasting and Late Onset neutropenia with and without antibodies against neutrophils. A potential new pathological entity?Dugotrajna neutropenija i neutropenija s kasnim početkom sa i bez antineutrofilnih protutijela: Potencijalno novi patološki entitet?Carlo Dufour1, Andrea Beccaria1, Francesca Fioredda11 Hematology Unit. Department of Hemato Oncology – IRCCS G. Gaslini Children’s Hospital, Genova, ItalyPregled | ReviewLate Onset autoimmune neutropenias (LOAIN) are forms of neutropenia associated to detectable serum (indirect) anti neutrophil antibodies that rise beyond the age of 3 years. Long Lasting autoimmune neutro-penias (LLAIN)are described as pediatric forms that do not remit within the classical time span of 24-36 months. LO/LL AIN were recently showed to have a different infectious pattern and a more pronounced autoimmune signature compared with primary auto-immune neutropenias (pAIN) that classically appear in the first year and remit within the third year of life and are associated to indirect positive anti neutrophil antibodies.When no anti neutrophil antibodies are detected in the serum the LO/LL neutropenias are defined as idi-opathic neutropenias (IN).In the present study we analyzed, a large cohort of LO/LL AIN and IN patients included in the Italian Neutropenia Registry (INR) by performing extensive clinical, hematological, immunological investigations, and genetic analysis aiming to see whether there were differences between LO/LL AIN and IN or if they shared clinical, hematological and immunological fea-tures signature and a genetic background.Indeed no major differences were detected between the two cohorts. A tendency to develop autoimmune phenomena was observed overtime in more than one third of the whole cohort. Specific alterations of B cells and T regulatory cells were observed in the cohort and pathogenic variants related to genes implicated in  immune-dysregulations were found in a minority of patients.Overall these findings suggest that LO/LL AIN and IN may represent a similar entity in which reduction of specific B cell subset and of T regulatory profile may predispose to development of autoimmune phenome-na that in some case rely on an immune-dysregulation genetic background.Young patients in whom autoimmune neutropenia does not remit in classical timespan should enter spe-cific immunological investigation and monitoring plans.

Dugotrajna neutropenija i neutropenija s kasnim početkom sa i bez antineutrofilnih protutijela: Potencijalno novi patološki entitet?

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