Résumé
The French national cohort of children with Langerhans cell histiocytosis (LCH) has included 1478 patients since it was established in 1983. LCH therapeutic strategies substantially changed in 1998, so we have divided the cohort into two 15-year periods. Starting in 1998, therapy duration increased from 6 to 12 months, repeated induction therapy was performed in cases showing a poor response to the first induction with vinblastine and steroids, and refractory disease in a risk organ (RO+) was treated with cladribine and cytarabine. A total of 483 (33%) patients were enrolled before 1998, and 995 (67%) after 1998. Five-year survival was 96·6% (95% confidence interval: 95·4–97·5%) overall, improving from 92% pre-1998 to 99% post-1998 (P < 0·001 adjusted to disease extent). This change was supported by an increase in 5-year survival from 60% to 92% in the RO+ group. Survival was particularly associated with cladribine and cytarabine among refractory RO+ patients. Disease reactivation was slightly less frequent after 1998, due to better enrolment of single-system patients, extended therapy duration, and more efficient second-line therapy. The crude rates of endocrine and neurological sequelae (the most frequent sequelae) appeared to improve over time, but this difference was not observed when the analysis was stratified by disease extent.
| langue originale | Anglais |
|---|---|
| Pages (de - à) | 887-898 |
| Nombre de pages | 12 |
| journal | British Journal of Haematology |
| Volume | 174 |
| Numéro de publication | 6 |
| Les DOIs | |
| état | Publié - 1 sept. 2016 |
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Dans: British Journal of Haematology, Vol 174, Numéro 6, 01.09.2016, p. 887-898.
Résultats de recherche: Contribution à un journal › Article › Revue par des pairs
TY - JOUR
T1 - Langerhans cell histiocytosis
T2 - therapeutic strategy and outcome in a 30-year nationwide cohort of 1478 patients under 18 years of age
AU - Rigaud, Charlotte
AU - Barkaoui, Mohamed A.
AU - Thomas, Caroline
AU - Bertrand, Yves
AU - Lambilliotte, Anne
AU - Miron, Jean
AU - Aladjidi, Nathalie
AU - Plat, Geneviève
AU - Jeziorski, Eric
AU - Galambrun, Claire
AU - Mansuy, Ludovic
AU - Lutz, Patrick
AU - Deville, Anne
AU - Armari-Alla, Corinne
AU - Reguerre, Yves
AU - Fraitag, Sylvie
AU - Coulomb, Aurore
AU - Gandemer, Virginie
AU - Leboulanger, Nicolas
AU - Moshous, Despina
AU - Hoang-Xuan, Khe
AU - Tazi, Abdellatif
AU - Heritier, Sébastien
AU - Emile, Jean François
AU - Donadieu, Jean
AU - Kebaili, Kamila
AU - Brugières, Laurence
AU - Mazingue, Françoise
AU - Pacquement, Hélène
AU - Mary, Pierre
AU - Jouve, Luc
AU - Journé, Alexandre
AU - Glorion, Christophe
AU - Coze, Carole
AU - Zerah, Michel
AU - Leverger, Guy
AU - Chastagner, Pascal
AU - Moine, Philippe
AU - Minckes, Odile
AU - Lutun, Anne
AU - Piguet, Christophe
AU - Blouin, Pascale
AU - Stephan, Louis
AU - Pellier, Isabelle
AU - Millot, Fréderic
AU - Kanold, Justyna
AU - Beaussant, Cohen
AU - Lescoeur, Brigitte
AU - Brousse, Nicole
AU - Haroche, Julien
AU - Genereau, Thierry
AU - Munzer, Martine
AU - Bugnet, Emmanuelle
AU - Couillault, Gerard
AU - Neven, Bénédicte
AU - Lepointe, Ducou
AU - Polak, Michel
AU - Rybojad, Michel
AU - Bodemer, Christine
AU - Debray, Dominique
AU - Devoldere, Catherine
AU - Li-Thiao-Te, Valerie
AU - Gourmel, Antoine
AU - Pautard, Brigitte
AU - Proust, Stephanie
AU - Brasme, Francois
AU - Rialland, Xavier
AU - Plouvier, Emmanuel
AU - Micheau, Magui
AU - Perel, Yves
AU - Verite, Cecile
AU - Ducassou, Stephane
AU - Carausu, Liana
AU - Deparis, Marianna
AU - Bodet, Damien
AU - Dore, Eric
AU - Demeocq, Francois
AU - Briandet, Claire
AU - Plantaz, Dominique
AU - Pagnier, Anne
AU - Adjaoud, Dalila
AU - Nelken, Brigitte
AU - Oudot, Caroline
AU - Berard, Perrine
AU - Dony, Arthur
AU - Philippe, Noel
AU - Gentet, Claude
AU - Rome, Angelique
AU - Herrmann, Iris
AU - Michel, Gerard
AU - Sirvent, Nicolas
AU - Tichit, Renaud
AU - Schmitt, Claudine
AU - Sommelet, Danielle
AU - Mechineau, Francoise
AU - Bellmann, Francoise
AU - Poiree, Maryline
AU - Soler, Christine
AU - Fasola, Sylvie
AU - Auvrignon, Anne
AU - Landman-Parker, Judith
AU - Petit, Arnaud
AU - Tabone, Dominique
AU - Debre, Marianne
AU - Blanche, Stephane
AU - Bader-Meunier, Brigitte
AU - Fischer, Alain
AU - Hamel, Dominique
AU - Cuinet, Aurelie
AU - Aerts, Isabelle
AU - Bourdeaut, Franck
AU - Doz, Francois
AU - Michon, Jean
AU - Orbach, Daniel
AU - Schleiermacher, Gudrun
AU - Minard, Veronique
AU - Valteau-Couanet, Dominique
AU - Kalifa, Chantal
AU - Blanc, Laurence
AU - Gorde, Stephanie
AU - Behar, Catherine
AU - Edan, Christine
AU - Bonneau, Jacinthe
AU - Chappe, Celine
AU - Taque, Sophie
AU - Toutain, Fabienne
AU - Dumesnil, Cecile
AU - Cardine, Aude
AU - Schneider, Pascale
AU - Vannier, Pierre
AU - Berger, Claire
AU - David, Audrey
AU - Thouvenin-Doulet, Sandrine
AU - Spiegel, Alexandra
AU - Robert, Alain
AU - Bertozzi, Isabelle
AU - Pasquet, Marlene
AU - Rubie, Herve
AU - Auvin, Cecile
AU - Colombat, Philippe
AU - Jourdain, Anne
AU - Yvert, Marion
AU - Jehanne, Mathile
AU - Stoven, Cecile
AU - Amoura, Zahir
AU - Cohen, Fleur
AU - Idbaih, Ahmed
AU - Lorillon, Gwenael
AU - De Menthon, Mathilde
AU - Mahr, Alfred
N1 - Publisher Copyright: © 2016 John Wiley & Sons Ltd
PY - 2016/9/1
Y1 - 2016/9/1
N2 - The French national cohort of children with Langerhans cell histiocytosis (LCH) has included 1478 patients since it was established in 1983. LCH therapeutic strategies substantially changed in 1998, so we have divided the cohort into two 15-year periods. Starting in 1998, therapy duration increased from 6 to 12 months, repeated induction therapy was performed in cases showing a poor response to the first induction with vinblastine and steroids, and refractory disease in a risk organ (RO+) was treated with cladribine and cytarabine. A total of 483 (33%) patients were enrolled before 1998, and 995 (67%) after 1998. Five-year survival was 96·6% (95% confidence interval: 95·4–97·5%) overall, improving from 92% pre-1998 to 99% post-1998 (P < 0·001 adjusted to disease extent). This change was supported by an increase in 5-year survival from 60% to 92% in the RO+ group. Survival was particularly associated with cladribine and cytarabine among refractory RO+ patients. Disease reactivation was slightly less frequent after 1998, due to better enrolment of single-system patients, extended therapy duration, and more efficient second-line therapy. The crude rates of endocrine and neurological sequelae (the most frequent sequelae) appeared to improve over time, but this difference was not observed when the analysis was stratified by disease extent.
AB - The French national cohort of children with Langerhans cell histiocytosis (LCH) has included 1478 patients since it was established in 1983. LCH therapeutic strategies substantially changed in 1998, so we have divided the cohort into two 15-year periods. Starting in 1998, therapy duration increased from 6 to 12 months, repeated induction therapy was performed in cases showing a poor response to the first induction with vinblastine and steroids, and refractory disease in a risk organ (RO+) was treated with cladribine and cytarabine. A total of 483 (33%) patients were enrolled before 1998, and 995 (67%) after 1998. Five-year survival was 96·6% (95% confidence interval: 95·4–97·5%) overall, improving from 92% pre-1998 to 99% post-1998 (P < 0·001 adjusted to disease extent). This change was supported by an increase in 5-year survival from 60% to 92% in the RO+ group. Survival was particularly associated with cladribine and cytarabine among refractory RO+ patients. Disease reactivation was slightly less frequent after 1998, due to better enrolment of single-system patients, extended therapy duration, and more efficient second-line therapy. The crude rates of endocrine and neurological sequelae (the most frequent sequelae) appeared to improve over time, but this difference was not observed when the analysis was stratified by disease extent.
KW - cohort study
KW - historical comparisons
KW - langerhans cell histiocytosis
UR - http://www.scopus.com/inward/record.url?scp=85027951421&partnerID=8YFLogxK
U2 - 10.1111/bjh.14140
DO - 10.1111/bjh.14140
M3 - Article
C2 - 27273725
AN - SCOPUS:85027951421
SN - 0007-1048
VL - 174
SP - 887
EP - 898
JO - British Journal of Haematology
JF - British Journal of Haematology
IS - 6
ER -