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Br J Haematol. 2016 Sep;174(6):887-98. doi: 10.1111/bjh.14140. Epub 2016 Jun 7.

Langerhans cell histiocytosis: therapeutic strategy and outcome in a 30-year nationwide cohort of 1478 patients under 18 years of age.

Author information

1
Registre français des histiocytoses, Service d'Hémato-oncologie Pédiatrique Hôpital Trousseau, AP-HP, Paris, France.
2
Service de Pédiatrie Institut Gustave Roussy, IGR, Villejuif.
3
Service d'hémato-oncologie pédiatrique, Centre Hospitalo-Universitaire de Nantes, Nantes, France.
4
Institut d'Hématologie et d'Oncologie Pédiatrique, Lyon, France.
5
Unité d'Hémato-oncologie Pédiatrique, Centre Hospitalo-Universitaire de Lille, Lille, France.
6
CIC 1401, Inserm CICP, Hopital Pellegrin-enfant, CEREVANCE, CHU de Bordeaux, Bordeaux, France.
7
Service d'hémato-oncologie pédiatrique, Centre Hospitalo-Universitaire de Toulouse, Toulouse, France.
8
Service d'hémato et d'oncologie pédiatrique, Centre Hospitalo-Universitaire de Montpellier, Montpellier, France.
9
Service d'Hématologie Oncologie pédiatrique, Centre Hospitalo-Universitaire de Marseille, Marseille, France.
10
Medecine Infantile 2, Centre Hospitalo-Universitaire de Nancy, Nancy, France.
11
Service d'hémato-oncologie pédiatrique, Centre Hospitalo-Universitaire de Strasbourg, Strasbourg, France.
12
Service d'hémato-oncologie pédiatrique, Centre Hospitalo-Universitaire de Nice, Nice, France.
13
Service d'hémato-oncologie pédiatrique, Centre Hospitalo-Universitaire de Grenoble, La Tronche, France.
14
Service d'hémato-oncologie pédiatrique, Centre Hospitalo-Universitaire de la Réunion Hôpital Felix Guyon, Saint denis, La Réunion, France.
15
Service d'Anatomopathologie, Hôpital Necker Enfants, AP-HP, Paris, France.
16
Service d'Anatomopathologie, Hopital Trousseau, Paris, France.
17
Service d'Hématologie pédiatrique, Centre Hospitalo-Universitaire de Rennes, Rennes, France.
18
Service d'Otorhinolaryngologie et Chirurgie Cervicofaciale, Hôpital Necker Enfants Malades, AP-HP, Paris, France.
19
Unité d'Immuno-hématologie pédiatrique, Hôpital Necker Enfants Malades, AP-HP, Paris, France.
20
Service de Neurologie, Groupe hospitalier Pitié Salpêtrière-Charles Foix, AP-HP, Sorbonne Universités - UPMC, Paris, France.
21
APHP Service de Pneumologie Centre de référence des histiocytoses Hôpital Saint Louis, Paris, France.
22
Service de pathologie, Hôpital Ambroise Paré, UVSQ, Université Paris-Saclay, AP-HP, Boulogne, France.

Abstract

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.

KEYWORDS:

cohort study; historical comparisons; langerhans cell histiocytosis

PMID:
27273725
DOI:
10.1111/bjh.14140
[Indexed for MEDLINE]

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