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2023EHA共识指南:成人和儿童中性粒细胞减少症的诊断和治疗

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2023EHA共识指南:成人和儿童中性粒细胞减少症的诊断和治疗-医知素材库
2023EHA共识指南:成人和儿童中性粒细胞减少症的诊断和治疗
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HemaSphereEHAEUROPEANHEMATOLOGYASSOCIATIONGuideline Article -Consensus basedOpen AccessThe European Guidelines on Diagnosis andManagement of Neutropenia in Adults andChildren:A Consensus Between theEuropean Hematology Association and theEuNet-INNOCHRON COST ActionFrancesca Fioredda',Julia Skokowa2,Hannah Tamary34,Michail Spanoudakiss,Piero Farruggia,Antonio Almeida,Daniela Guardo',Petter Hoglund9.10.11,Peter E.Newburger2,Jan Palmblad.11,Ivo P.Touw3,Cornelia Zeidler14,Alan J.Warren15.16.17,David C.Dale',Karl Welte19,Carlo Dufour',Helen A.Papadaki20.21Correspondence:Francesca Fioredda(francescafioredda@gaslini.org);Helen A.Papadaki (e.papadaki@uoc.gr).ABSTRACTNeutropenia,as an isolated blood cell deficiency,is a feature of a wide spectrum of acquired or congenital,benign or premalignant dis-orders with a predisposition to develop myelodysplastic neoplasms/acute myeloid leukemia that may arise at any age.In recent years,advances in diagnostic methodologies,particularly in the field of genomics,have revealed novel genes and mechanisms responsible foretiology and disease evolution and opened new perspectives for tailored treatment.Despite the research and diagnostic advances inthe field,real world evidence,arising from international neutropenia patient registries and scientific networks,has shown that the diag-nosis and management of neutropenic patients is mostly based on the physicians'experience and local practices.Therefore,expertsparticipating in the European Network for the Innovative Diagnosis and Treatment of Chronic Neutropenias have collaborated under theauspices of the European Hematology Association to produce recommendations for the diagnosis and management of patients acrossthe whole spectrum of chronic neutropenias.In the present article,we describe evidence-and consensus-based guidelines for thedefinition and classification,diagnosis,and follow-up of patients with chronic neutropenias including special entities such as pregnancyand the neonatal period.We particularly emphasize the importance of combining the clinical findings with classical and novel laboratorytesting,and advanced germline and/or somatic mutational analyses,for the characterization,risk stratification,and monitoring of theentire spectrum of neutropenia patients.We believe that the wide clinical use of these practical recommendations will be particularlybeneficial for patients,families,and treating physicians.Unit of Hematobgy,IRCCS Istituto Giannina Gasini Genova,ltalyDeperment of Hematology University of Cambridge,Unied KingdomDepartment of Oncology,Hematology,Immunology,Rheumatology,and ClinicalCambridge Institute for Medical Research,University of Cambridge,UnitedImmunobgy,University Hospital Tubingen,GermanyKngdomThe Rina Zaizov HematologyOncology Division,Schneider Children's MedicalWelcome Trust-Medical Research Council Stem Cell Institute,University ofCenter of Israel,Petah Tikva,IsraelCambridge,United KingdomSackler School of Medicine,Tel Aviv University Israel
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