loading page

ELANE neutropenia and solid tumors: 4 cases from the French severe chronic neutropenia registry
  • +11
  • Jean Donadieu,
  • Fares BOU MITRI,
  • Blandine BEAUPAIN,
  • nathalie Aladjidi,
  • jean-françois VIALLARD,
  • jean-Philippe LE PAIH,
  • mokrane yacoub,
  • Thierry Leblanc,
  • laurent quero,
  • alexia rouland,
  • louis LABBE,
  • claire deback,
  • Christine Bellanné-Chantelot,
  • Jean-Francois EMILE
Jean Donadieu
CHU Trousseau

Corresponding Author:jean.donadieu@aphp.fr

Author Profile
Fares BOU MITRI
Armand-Trousseau Childrens Hospital
Author Profile
Blandine BEAUPAIN
Armand-Trousseau Childrens Hospital
Author Profile
nathalie Aladjidi
University hospital of Bordeaux
Author Profile
jean-françois VIALLARD
CHU de Bordeaux
Author Profile
jean-Philippe LE PAIH
CHU Bordeaux GH Pellegrin
Author Profile
mokrane yacoub
CHU Bordeaux GH Pellegrin
Author Profile
Thierry Leblanc
Assistance Publique - Hôpitaux de Paris
Author Profile
laurent quero
Hopital Saint-Louis
Author Profile
alexia rouland
CHU Dijon
Author Profile
louis LABBE
hotel dieu Le creusot
Author Profile
claire deback
Universite Paris-Saclay Faculte des Sciences d'Orsay
Author Profile
Christine Bellanné-Chantelot
Hopital Universitaire Pitie Salpetriere
Author Profile
Jean-Francois EMILE
Ambroise Pare Hospital Group
Author Profile

Abstract

Neutropenia related to ELANE gene mutations predisposes to infection and leukemia/ myelodysplasia, but little is known about the predisposition to cancer. Among a cohort of 149 patients, we identified four with malignant solid tumors (papillary thyroid cancer, anal squamous cell cancer, papillary renal cell carcinoma, and adrenocortical carcinoma), all after the age of 25 years. Three occurred in cyclic neutropenia while 1 occurred in severe chronic neutropenia (among 49 and 100 patients, respectively). A previous radiotherapy was identified as risk factor in one patient. Moreover, among 18 other patients that underwent hematopoietic stem-cell transplantations, none developed a cancer.