Reactivations in multisystem Langerhans cell histiocytosis: data of the international LCH registry.
J Pediatr
; 153(5): 700-5, 705.e1-2, 2008 Nov.
Article
em En
| MEDLINE
| ID: mdl-18589441
ABSTRACT
OBJECTIVE:
To assess multisystem Langerhans cell histiocytosis reactivation and its impact on morbidity and mortality. STUDYDESIGN:
Retrospective analysis of 335 patients with MS-LCH and documented complete disease resolution (NAD1).RESULTS:
The probability of a reactivation within 5 years of NAD1 was 46%. The first reactivation occurred within 2 years after NAD1 in most of the patients. Of 134 events, 35% were confined to skeleton, 24% were single-system nonbony lesions, 24% were multisystem reactivations without risk-organ involvement, and 10% with risk-organ involvement. In 7%, the location was unspecified. Only 3 deaths (2.2%) were documented within the context of a first reactivation. Second disease resolution (NAD2) was achieved in 85% of the cases. The probability of a second reactivation within 5 years of NAD2 was 44%. The risk for permanent consequences in patients with reactivations was higher, compared with patients without reactivation (RHR 2.2, P = .046).CONCLUSIONS:
Reactivation is a frequent and early event in MS-LCH, but involvement of risk organs at reactivation is rare and mortality is minimal. However, reactivations increase the risk for permanent consequences by about 2-fold. Prospective trials targeting reduction of acute morbidity and permanent disabilities through nontoxic treatment of the reactivations are warranted.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Histiocitose de Células de Langerhans
Idioma:
En
Ano de publicação:
2008
Tipo de documento:
Article