Recurrent bacterial infections, but not fungal infections, characterise patients with ELANE-related neutropenia: a French Severe Chronic Neutropenia Registry study.
ELANE-related neutropenia
opportunistic infections
severe congenital neutropenia
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
09 2021
09 2021
Historique:
received:
19
04
2021
accepted:
21
06
2021
pubmed:
3
8
2021
medline:
16
12
2021
entrez:
2
8
2021
Statut:
ppublish
Résumé
Among 143 patients with elastase, neutrophil-expressed (ELANE)-related neutropenia enrolled in the French Severe Chronic Neutropenia Registry, 94 were classified as having severe chronic neutropenia (SCN) and 49 with cyclic neutropenia (CyN). Their infectious episodes were classified as severe, mild or oral, and analysed according to their natural occurrence without granulocyte-colony stimulating factor (G-CSF), on G-CSF, after myelodysplasia/acute leukaemia or after haematopoietic stem-cell transplantation. During the disease's natural history period (without G-CSF; 1913 person-years), 302, 957 and 754 severe, mild and oral infectious events, respectively, occurred. Among severe infections, cellulitis (48%) and pneumonia (38%) were the most common. Only 38% of episodes were microbiologically documented. The most frequent pathogens were Staphylococcus aureus (37·4%), Escherichia coli (20%) and Pseudomonas aeruginosa (16%), while fungal infections accounted for 1%. Profound neutropenia (<200/mm
Substances chimiques
ELANE protein, human
EC 3.4.21.37
Leukocyte Elastase
EC 3.4.21.37
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
908-920Investigateurs
Elodie Gouache
(E)
Fanny Alby-Laurent
(F)
Yves Bertrand
(Y)
Pierre-Simon Rohrlich
(PS)
Eric Jeziorksi
(E)
Liana Carausu
(L)
Marc Michel
(M)
Valérie L T Te
(VLT)
Philippe Bensaid
(P)
Loïc de Pontual
(L)
Louis Terriou
(L)
Nicolas Schleinitz
(N)
Karima Yakouben
(K)
Nizar Mahlaoui
(N)
Felipe Suarez
(F)
Xavier Delbrel
(X)
Dalila Adjaoud
(D)
Jean-Louis Stephan
(JL)
Guy Leverger
(G)
Fanny Fouyssac
(F)
Alice Garnier
(A)
Chiara Sileo
(C)
Aline Moignet
(A)
Nathalie Cheikh
(N)
Damien Bodet
(D)
Eric Dore
(E)
Florent Neumann
(F)
Fabrice Jardin
(F)
Informations de copyright
© 2021 British Society for Haematology and John Wiley & Sons Ltd.
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