The Journal of pediatrics · 2008
Reactivations in multisystem Langerhans cell histiocytosis: data of the international LCH registry.
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Minkov Milen, Steiner Manuel, Pötschger Ulrike, Aricò Maurizio, Braier Jorge, Donadieu Jean, Grois Nicole, Henter Jan-Inge, Janka Gritta, McClain Kenneth, Weitzman Sheila, Windebank Kevin, Ladisch Stephan, Gadner Helmut, International LCH Study Group
Аннотация
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.
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.