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. / Minkov, Milen; Steiner, Manuel; Pötschger, Ulrike; Aricò, Maurizio; Braier, Jorge; Donadieu, Jean; Grois, Nicole; Henter, Jan-Inge; Janka-Schaub, Gritta; McClain, Kenneth; Weitzman, Sheila; Windebank, Kevin; Ladisch, Stephan; Gadner, Helmut.
In: J PEDIATR-US, Vol. 153, No. 5, 5, 2008, p. 1-2, 700-705.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Reactivations in multisystem Langerhans cell histiocytosis: data of the international LCH registry.
AU - Minkov, Milen
AU - Steiner, Manuel
AU - Pötschger, Ulrike
AU - Aricò, Maurizio
AU - Braier, Jorge
AU - Donadieu, Jean
AU - Grois, Nicole
AU - Henter, Jan-Inge
AU - Janka-Schaub, Gritta
AU - McClain, Kenneth
AU - Weitzman, Sheila
AU - Windebank, Kevin
AU - Ladisch, Stephan
AU - Gadner, Helmut
PY - 2008
Y1 - 2008
N2 - 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.
AB - 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.
M3 - SCORING: Zeitschriftenaufsatz
VL - 153
SP - 1-2, 700-705
JO - J PEDIATR-US
JF - J PEDIATR-US
SN - 0022-3476
IS - 5
M1 - 5
ER -
