Outcomes for patients with severe chronic neutropenia treated with granulocyte colony-stimulating factor.

Autor:	Dale DC; Department of Medicine, and., Bolyard AA; Severe Chronic Neutropenia International Registry, University of Washington, Seattle, WA., Shannon JA; Severe Chronic Neutropenia International Registry, University of Washington, Seattle, WA., Connelly JA; Division of Hematology-Oncology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN., Link DC; Section of Stem Cell Biology, Division of Oncology, Washington University School of Medicine, St. Louis, MO., Bonilla MA; Pediatric Hematology/Oncology, St. Joseph's Children's Hospital, Paterson, NJ; and., Newburger PE; Department of Pediatrics, University of Massachusetts Medical School, Worcester, MA.
Jazyk:	angličtina
Zdroj:	Blood advances [Blood Adv] 2022 Jul 12; Vol. 6 (13), pp. 3861-3869.
DOI:	10.1182/bloodadvances.2021005684
Abstrakt:	Severe chronic neutropenia (SCN), defined as blood neutrophils <0.5 × 109/L for >3 months, is an uncommon hematological condition associated with recurrent and severe bacterial infections. After short-term clinical trials showed the benefits of granulocyte colony-stimulating factor (G-CSF) treatment for SCN, SCNIR (Severe Chronic Neutropenia International Registry) opened to determine the long-term benefits and safety of this treatment. This report summarizes findings from more than 16 000 patient-years of prospective observations for patients with congenital and acquired SCN. We observed that adverse outcomes depend on the underlying etiology. Myelodysplasia (MDS) and acute myeloid leukemia (AML) occur infrequently and largely in patients with congenital neutropenias. Having cyclic or chronic autoimmune/ idiopathic neutropenia portends a favorable prognosis. A few patients with idiopathic neutropenia evolve to develop lymphoid malignancies, but they do not appear to be at increased risk of myeloid malignancies, even with very long-term G-CSF therapy. Progression to systemic autoimmune diseases, bone marrow (BM) failure, aplastic anemia, or nonmyeloid malignancies are not expected consequences of SCN or treatment with G-CSF. (© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)
Databáze:	MEDLINE
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