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Blood. 2015 Oct 1;126(14):1643-50. doi: 10.1182/blood-2015-03-634493. Epub 2015 Aug 10.

Severe chronic primary neutropenia in adults: report on a series of 108 patients.

Collaborators (259)

Gourmel A, Devoldere C, Thiao V, Lutun A, Rialland X, Pellier I, Rachieru I, Brasme J, Bensaid P, Ifrah N, Gardembas Pain M, Francois S, Boyer Perrard F, Hunault Berger M, Schmidt A, Delorme C, Bauduer F, Plouvier E, Beaussant Cohen S, Deconinck E, Daltroff G, Borm B, Palenzuela G, Guitton C, Goujard C, De Pontual L, Perel Y, Micheau M, Aladjidi N, Verite C, Lacombe D, Taieb A, Viallard J, Lemoine A, Carusu L, Ansquer H, Berthou P, Poirel A, Cassassus P, Minckes O, Bodet D, Deparis M, Damaj G, Reman O, Labrune P, Gadjos V, Perry A, Trioche P, Demeocq F, Kanold J, Merlin E, Dore E, Borderon C, Bay Jo, Tournilhac O, Bouscary D, Godeau B, Michel M, Lelievre J, Croisille L, Couillaud G, Briandet C, Faivre L, Thauvin C, Aral B, Wetterwald M, Hatchuel Y, Gutnecht J, Cahn J, Bouillet L, Chouraqui J, Plantaz D, Armari C, Adjaoud D, Pagnier A, Delion, Layadi M, Zairi E, Reguerre Y, Jehanne M, Boumahni B, Sanyas P, Morel P, Dupriez B, Besancon A, Martin Coignard D, Lefevre G, Catteau B, Nelken B, Mazingue F, Bruno B, Lambilliote A, Abouchala W, Gottrand F, Demory J, Bordesoules D, Oudot C, Piguet E, Debourdeau P, Lachaux A, Le Gall L, Guffon N, Bertrand Y, Renard C, Kebaili K, Nove Josserand L, Michel G, Barlogis V, Thuret I, Galambrun C, Chambost H, Sarles J, Roquelaure B, Stoppa A, Charbonnier A, Kaplanski G, Schleinitz N, Harle J, Gouraud F, Rouquier Thisse F, Dorvaux V, Jeziorski E, Sirvent N, Haouy S, Rivier F, Sarda P, Pinson L, Rieu D, Schved J, Lamour A, Drenou B, Benoit M, Ginglinger E, Latger V, Mansuy L, Chastagner P, Fouyssac F, Chabot J, Ranta D, Perrot A, Leheup B, Feillet F, Bronowicki J, Romefort B, Moreau P, Isidor B, Neel A, Thomas C, Rialland F, Strullu M, Audrain M, Fischer A, Blanche S, Picard C, Mahlaoui N, Neven B, Moshous D, Rummele F, Talbotec C, Goullet O, Lacaille F, De Lonlay P, Bonnet D, Rio M, Macintyre E, Hermine O, Varet B, Suarez F, Monpoux F, Deville A, Poiree M, Soler C, Rorlich P, Monceaux F, Perdereau S, Schoenwald M, Delbrel X, Leblond V, Pellegrino B, Lachenaud M, Millot F, Blayo M, Fenneteau O, Yakouben K, Dalle J, Ouachee M, Lescoeur B, Baruchel A, Brethon B, Bellaiche M, Gordes Jean S, Gandemer V, Bayart S, Toutain F, Dabadie A, Lamy De La Chapelle T, Dauriac F, Nimubona S, Plantier I, Vannier J, Schneider P, Marie Cardine A, Dumesnil C, Jardin F, Fain O, Coppo P, Garderet L, Mohty M, Berger C, Stephan Jl, Gay C, Oksenhendler E, Fieschi C, Fermand J, Galicier L, Borie R, Raffoux E, Dombret H, Socie G, Peffault De La Tour R, Sicre De Fontbrune F, Boissel N, Lengline E, Fenaux P, Lutz Jp, Paillard C, Bergerat Jp, Herbrecht R, Maloisel F, Lioure B, Pasquali J, Rubie H, Plat G, Pasquet M, Attal M, Recher C, Broue P, Colombat P, Lejars O, Blouin P, Yvert M, Labarthe F, Hoarau C, Dine G, Manteau C, Dollfus C, Donadieu J, Landman J, Leverger G, Auvrignon A, Tabone M, Petit J, Fasola S, Favier R, Lapillonne H, Ballerini P, Tounian P, Dubern B, Cagnard B.

Author information

1
Service d'Hématologie-Greffe, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France;
2
Département d'Hématologie Clinique, Hôpital Universitaire de Rennes, Rennes, France, and INSERM U1414-CIC, Université Rennes I;
3
Service d'Hématologie Pédiatrique, Hôpital Trousseau, Assistance Publique Hôpitaux de Paris, Paris, France;
4
Service d'Hématologie Adulte, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France;
5
Service d'Immunologie Clinique, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France;
6
Service d'Hématologie, Hôpital Saint Antoine, Assistance Publique Hôpitaux de Paris, Paris, France;
7
Service de Médecine Interne and.
8
Service d'Hématologie, Hôpital Henri Mondor, Assistance Publique Hôpitaux de Paris, Paris, France;
9
Service d'Hématologie Adulte, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France;
10
Service d'Hématologie, Centre Hospitalier Régional Universitaire de Lille, Lille, France;
11
Service d'Hématologie, Hôpital Hôtel-Dieu, Centre Hospitalier Régional Universitaire de Nantes, Nantes, France;
12
Service d'Hématologie Clinique, Centre Hospitalier Universitaire de Caen, Caen, France;
13
Service de Médecine Interne, Hôpital Kremlin-Bicêtre, Assistance Publique Hôpitaux de Paris, Kremlin-Bicêtre, France;
14
Service des Maladies du Sang, Centre Hospitalier Universitaire Angers, Angers, France;
15
Service d'Hématologie, Hôpital Lyon-Sud, Hospices Civiles de Lyon, Lyon, France;
16
Laboratoire d'Immunologie Leuco-plaquettaire et d'Histocompatibilité, Hôpital Henri Mondor, Etablissement Français du Sang d'Ile de France, Créteil, France;
17
Laboratoire d'Immunologie, Institut de Biologie, Centre Hospitalier Universitaire de Nantes, Nantes, France; and.
18
Département de Génétique, Hôpital de la Pitié-Salpêtrière, Université Pierre et Marie Curie, Assistance Publique Hôpitaux de Paris, Paris, France.

Abstract

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.

PMID:
26261239
DOI:
10.1182/blood-2015-03-634493
[Indexed for MEDLINE]
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