Severe chronic primary neutropenia in ...
Type de document :
Article dans une revue scientifique: Article original
PMID :
URL permanente :
Titre :
Severe chronic primary neutropenia in adults: report on a series of 108 patients
Auteur(s) :
Sicre De Fontbrune, Flore [Auteur]
Moignet, Aline [Auteur]
Beaupain, Blandine [Auteur]
Suarez, Felipe [Auteur]
Galicier, Lionel [Auteur]
Socie, Gerard [Auteur]
Varet, Bruno [Auteur]
Coppo, Paul [Auteur]
Michel, Marc [Auteur]
Pautas, Cecile [Auteur]
Oksenhendler, Eric [Auteur]
Lengline, Etienne [Auteur]
Terriou, Louis [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Lille Inflammation Research International Center - U 995 [LIRIC]
Moreau, Philippe [Auteur]
Chantepie, Sylvain [Auteur]
Casadevall, Nicole [Auteur]
Michot, Jean Marie [Auteur]
Gardembas, Martine [Auteur]
Michallet, Mauricette [Auteur]
Croisille, Laure [Auteur]
Audrain, Marie [Auteur]
Bellanne-Chantelot, Christine [Auteur]
Donadieu, Jean [Auteur]
Lamy, Thierry [Auteur]
Moignet, Aline [Auteur]
Beaupain, Blandine [Auteur]
Suarez, Felipe [Auteur]
Galicier, Lionel [Auteur]
Socie, Gerard [Auteur]
Varet, Bruno [Auteur]
Coppo, Paul [Auteur]
Michel, Marc [Auteur]
Pautas, Cecile [Auteur]
Oksenhendler, Eric [Auteur]
Lengline, Etienne [Auteur]
Terriou, Louis [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Lille Inflammation Research International Center - U 995 [LIRIC]
Moreau, Philippe [Auteur]
Chantepie, Sylvain [Auteur]
Casadevall, Nicole [Auteur]
Michot, Jean Marie [Auteur]
Gardembas, Martine [Auteur]
Michallet, Mauricette [Auteur]
Croisille, Laure [Auteur]
Audrain, Marie [Auteur]
Bellanne-Chantelot, Christine [Auteur]
Donadieu, Jean [Auteur]
Lamy, Thierry [Auteur]
Titre de la revue :
Blood
Nom court de la revue :
Blood
Numéro :
126
Pagination :
1643-1650
Date de publication :
2015-10-01
ISSN :
0006-4971
Discipline(s) HAL :
Sciences du Vivant [q-bio]
Résumé en anglais : [en]
Severe chronic primary neutropenia (CPN) is a rare entity, and long-term outcome and risk factors for infections in severe CPN adults have not been described to date. We report the characteristics and outcomes of 108 severe ...
Lire la suite >Severe chronic primary neutropenia (CPN) is a rare entity, and long-term outcome and risk factors for infections in severe CPN adults have not been described to date. We report the characteristics and outcomes of 108 severe adult CPN patients enrolled in a multi-institutional observational study. Severe CPN adults were mostly female (78%), and median age at diagnosis was 28.3 years. Diagnosis was fortuitous in 62% of cases. The median absolute neutrophil count (ANC) at diagnosis was 0.4 × 10(9)/L, and median ANC without granulocyte colony-stimulating factor (G-CSF) during follow-up was 0.5 × 10(9)/L. Twenty-three of 66 (34.8%) evaluable patients had neutrophil autoantibodies, and 6 of 47 (12.8%) a T-cell clone. The presence of neutrophil autoantibodies or T-cell clone was not associated with any specific clinical or biological characteristics. No death or hematologic malignancies occurred, and 44 severe bacterial infections were reported in 27 patients with a median follow-up of 8.3 years. Fifty patients received G-CSF either sporadically (n = 24) or continuously (n = 26) and responded (96%). Nineteen patients received immunosuppressive therapies: overall response (OR) was 41%, and median duration of response was 3 months. At diagnosis, the only predictive factor for the occurrence of severe bacterial infections was an ANC count below 0.2 × 10(9)/L (OR, 0.76). Severe CPN in adults is characterized by a female predominance and a benign outcome with a low rate of severe bacterial infections and no secondary malignancies. G-CSF is efficient and well tolerated but is not required in a majority of patients.Lire moins >
Lire la suite >Severe chronic primary neutropenia (CPN) is a rare entity, and long-term outcome and risk factors for infections in severe CPN adults have not been described to date. We report the characteristics and outcomes of 108 severe adult CPN patients enrolled in a multi-institutional observational study. Severe CPN adults were mostly female (78%), and median age at diagnosis was 28.3 years. Diagnosis was fortuitous in 62% of cases. The median absolute neutrophil count (ANC) at diagnosis was 0.4 × 10(9)/L, and median ANC without granulocyte colony-stimulating factor (G-CSF) during follow-up was 0.5 × 10(9)/L. Twenty-three of 66 (34.8%) evaluable patients had neutrophil autoantibodies, and 6 of 47 (12.8%) a T-cell clone. The presence of neutrophil autoantibodies or T-cell clone was not associated with any specific clinical or biological characteristics. No death or hematologic malignancies occurred, and 44 severe bacterial infections were reported in 27 patients with a median follow-up of 8.3 years. Fifty patients received G-CSF either sporadically (n = 24) or continuously (n = 26) and responded (96%). Nineteen patients received immunosuppressive therapies: overall response (OR) was 41%, and median duration of response was 3 months. At diagnosis, the only predictive factor for the occurrence of severe bacterial infections was an ANC count below 0.2 × 10(9)/L (OR, 0.76). Severe CPN in adults is characterized by a female predominance and a benign outcome with a low rate of severe bacterial infections and no secondary malignancies. G-CSF is efficient and well tolerated but is not required in a majority of patients.Lire moins >
Langue :
Anglais
Audience :
Internationale
Vulgarisation :
Non
Établissement(s) :
CHU Lille
Inserm
Université de Lille
Inserm
Université de Lille
Collections :
Date de dépôt :
2019-12-09T16:52:29Z