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

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.

Consensus of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) and the Brazilian Ministry of Health - General management of blood and blood products on the tests necessary for the release of exceptional medicines for sickle cell disease

“…neutrophils below 1000 cells/μL in babies up to one year of age and below 1500 cells/μL in over one-year-old patients 10 …”

“…Contraindications for taking HU : neutrophils below 1000 cells/μL in babies up to one year of age and below 1500 cells/μL in over one-year-old patients 10 Hb below 4.5 g/dL Reticulocyte count below 80,000/mm 3 (when Hb is <8 g/dL) Platelet count below 80,000/mm 3 or Pregnancy …”

Differential transcriptional control of hematopoiesis in congenital and cycling neutropenia patients harboring <i>ELANE</i> mutations

“…BM samples of CN and CyN patients were collected in accordance with an annual follow-up recommendation of the SCNIR and the EHA. 38 The study was conducted under the approval of the Ethical Board of the Medical Faculty, University of Tübingen. Written informed consent was obtained from all participants.…”