Reactivations in Multisystem Langerhans Cell Histiocytosis: Data of the International LCH Registry - 07/03/14
International LCH Study Group
Résumé |
Objective |
To assess multisystem Langerhans cell histiocytosis reactivation and its impact on morbidity and mortality.
Study design |
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.
Le texte complet de cet article est disponible en PDF.Abbreviations : DI, LCH, SS-LCH, MS-LCH, RO+, RO−, NAD1, NAD2, PC, R1, R2, SS-R, MS-R
Plan
The authors declare no real or perceived conflicts of interest. |
Vol 153 - N° 5
P. 700 - novembre 2008 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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