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. 2008 Nov;153(5):700-5, 705.e1-2.
doi: 10.1016/j.jpeds.2008.05.002. Epub 2008 Jun 27.

Reactivations in Multisystem Langerhans Cell Histiocytosis: Data of the International LCH Registry

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Reactivations in Multisystem Langerhans Cell Histiocytosis: Data of the International LCH Registry

Milen Minkov et al. J Pediatr. .

Abstract

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.

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