Chronic neutropenia: how best to assess severity and approach management?

被引:8
作者
Donadieu, Jean [1 ]
Frenz, Stephanie [2 ]
Merz, Lauren [3 ]
Sicre De Fontbrune, Flore [4 ]
Rotulo, Gioacchino Andrea [5 ]
Beaupain, Blandine [1 ]
Biosse-Duplan, Martin [6 ]
Audrain, Marie [7 ]
Croisille, Laure [8 ]
Ancliff, Phil [9 ]
Klein, Christoph [2 ]
Bellanne-Chantelot, Christine [10 ]
机构
[1] Hop Armand Trousseau, AP HP, Ctr Reference Neutropenies Chron, Registre Natl Neutropenies Congenitales,Serv Hema, 26,Ave Dr Netter, F-75012 Paris, France
[2] Ludwig Maximilians Univ Munchen, Dr Von Hauner Childrens Hosp, Dept Pediat, Univ Hosp, Munich, Germany
[3] Brigham & Womens Hosp, Dept Internal Med, 75 Francis St, Boston, MA 02115 USA
[4] Hop St Louis, APHP, Serv Hematol Greffe, Paris, France
[5] Univ Genoa, Dept Neurosci Rehabil Ophthalmol Genet Maternal &, Genoa, Italy
[6] Hop Bretonneau, AP HP, Serv Med Bucco Dentaire, Paris, France
[7] Serv Immunol Lab Biol, Chu Nantes, 9 Quai Moncousu, Bagneux, France
[8] Lab Hla ilp Efs Ile France 1, Creteil, France
[9] Great Ormond St Hosp London, Pediat Hematol, London, England
[10] Sorbonne Univ, Dept Genet Med, Hop Pitie Salpetriere, AP HP, Paris, France
关键词
Neutropenia; children; gcsf; diagnostic evaluation; classification; SEVERE CONGENITAL NEUTROPENIA; AUTOIMMUNE NEUTROPENIA; LONG-TERM; HEMATOLOGIC COMPLICATIONS; INFECTIOUS COMPLICATIONS; IDIOPATHIC NEUTROPENIA; NEONATAL NEUTROPENIA; CSF3R MUTATIONS; WHIM SYNDROME; RISK-FACTORS;
D O I
10.1080/17474086.2021.1976634
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Introduction Neutropenia is a relatively common finding in medical practice and the medical approach requires a gradual and pertinent diagnostic procedure as well as adapted management. Areas Covered The area of chronic neutropenia remains fragmented between diverse diseases or situations. Here physicians involved in different aspects of chronic neutropenia gather both the data from medical literature till the end of May 2021 and their experience to offer a global approach for the diagnosis of chronic neutropenia as well as their medical care. Expert opinion In most cases, the neutropenia is transient, frequently related to a viral infection, and not harmful. However, neutropenia can be chronic (i.e. >3 months) and related to a number of etiologies, some clinically benign, such as so-called 'ethnic' neutropenia. Autoimmune neutropenia is the common form in young children, whereas idiopathic/immune neutropenia is a frequent etiology in young females. Inherited neutropenia (or congenital neutropenia) is exceptional, with approximately 30 new cases per 10(6) births and 30 known subtypes. Such patients have a high risk of invasive bacterial infections, and oral infections. Supportive therapy, which is primarily based on daily administration of an antibiotic prophylaxis and/or treatment with granulocyte-colony stimulating factor (G-CSF), contributes to avoiding recurrent infections.
引用
收藏
页码:945 / 960
页数:16
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