SEARCHGUIDELINEShematology, pediatrics

guidelines·hematology, pediatrics, obstetrics and gynecology, immunology, clinical trial·PMC10065839

The European Guidelines on Diagnosis and Management of Neutropenia in Adults and Children: A Consensus Between the European Hematology Association and the EuNet-INNOCHRON COST Action

HemaSphere · 17 authors, 21 centres

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TL;DR

An expert panel developed evidence- and consensus-based recommendations (Delphi approach, >75% agreement) for the definition, classification, diagnosis, follow-up, and management of chronic neutropenias across the full clinical spectrum in adults and children.

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A 17-member expert panel from EuNet-INNOCHRON and the European Hematology Association developed recommendations across five topics: definition/classification, diagnostic approach, natural history/follow-up, treatment, and special situations. Literature review comprised case-control and cohort studies for most topics; the treatment topic was reviewed separately by Cochrane Hematology due to higher-level randomized trial evidence. Consensus was achieved via the Delphi approach with agreement required from more than 75% of panelists. Genetic testing is recommended for patients with severe congenital neutropenia and recurrent infections or family history. Limitations include reliance predominantly on lower-level observational evidence for most topics and the exclusion of a treatment-specific evidence review from this document.

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guidelines

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hematology

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ORIGINAL PAPER

PMC10065839

The European Guidelines on Diagnosis and Management of Neutropenia in Adults and Children: A Consensus Between the European Hematology Association and the EuNet-INNOCHRON COST Action

HemaSphere

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ABSTRACT

Neutropenia, as an isolated blood cell deficiency, is a feature of a wide spectrum of acquired or congenital, benign or premalignant disorders 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 for etiology and disease evolution and opened new perspectives for tailored treatment. Despite the research and diagnostic advances in the field, real world evidence, arising from international neutropenia patient registries and scientific networks, has shown that the diagnosis and management of neutropenic patients is mostly based on the physicians’ experience and local practices. Therefore, experts participating in the European Network for the Innovative Diagnosis and Treatment of Chronic Neutropenias have collaborated under the auspices of the European Hematology Association to produce recommendations for the diagnosis and management of patients across the whole spectrum of chronic neutropenias. In the present article, we describe evidence- and consensus-based guidelines for the definition and classification, diagnosis, and follow-up of patients with chronic neutropenias including special entities such as pregnancy and the neonatal period. We particularly emphasize the importance of combining the clinical findings with classical and novel laboratory testing, and advanced germline and/or somatic mutational analyses, for the characterization, risk stratification, and monitoring of the entire spectrum of neutropenia patients. We believe that the wide clinical use of these practical recommendations will be particularly beneficial for patients, families, and treating physicians.

CITATION EXPORT

Francesca Fioredda, Julia Skokowa, Hannah Tamary et al.. The European Guidelines on Diagnosis and Management of Neutropenia in Adults and Children: A Consensus Between the European Hematology Association and the EuNet-INNOCHRON COST Action. HemaSphere. (2023). https://doi.org/10.1097/HS9.0000000000000872

AUTHORS

17 · 21 CENTRES

Francesca Fioredda, Julia Skokowa, Hannah Tamary, Michail Spanoudakis, Piero Farruggia, Antonio Almeida, Daniela Guardo, Petter Höglund, Peter E. Newburger, Jan Palmblad, Ivo P. Touw, Cornelia Zeidler, Alan J. Warren, David C. Dale, Karl Welte, Carlo Dufour, Helen A. Papadaki

1Unit of Hematology, IRCCS Istituto Giannina Gaslini, Genova, Italy
2Department of Oncology, Hematology, Immunology, Rheumatology, and Clinical Immunology, University Hospital Tübingen, Germany
3The Rina Zaizov Hematology/Oncology Division, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel
4Sackler School of Medicine, Tel Aviv University, Israel
5Department of Hematology, Warrington and Halton Teaching Hospitals NHS foundation Trust, Warrington, United Kingdom
6Pediatric Onco-Hematology, ARNAS Civico Di Cristina Benfratelli Hospital, Palermo, Italy
7Department of Hematology, Hospital da Luz Lisboa, Portugal
8Faculdade de Medicina, Universidade Católica Portuguesa, Lisbon, Portugal
9Clinical Immunology and Transfusion Medicine Clinic, Karolinska University Hospital, Stockholm, Sweden
10Center for Hematology and Regenerative Medicine (HERM), Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden
11Department of Hematology, Karolinska University Hospital, Stockholm, Sweden
12Department of Pediatrics, UMass Chan Medical School, Worcester, MA, USA
13Department of Hematology and Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, Netherlands
14Department of Oncology, Hematology, Immunology and Bone Marrow Transplantation, Hannover Medical School, Hannover, Germany
15Department of Hematology, University of Cambridge, United Kingdom
16Cambridge Institute for Medical Research, University of Cambridge, United Kingdom
17Wellcome Trust–Medical Research Council Stem Cell Institute, University of Cambridge, United Kingdom
18University of Washington, Seattle, WA, USA
19University Children’s Hospital Tübingen, Germany
20Hemopoiesis Research Laboratory, School of Medicine, University of Crete, Heraklion, Greece
21Department of Hematology, University Hospital of Heraklion, Crete, Greece