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LCH-II (1996-2001)

LCH-II was a randomized phase-III trial for patients with multisystem LCH.1 It investigated the value of the addition of etoposide (Arm B) to the standard combination of prednisolone and vinblastine (Arm A) in patients with MS-LCH. The randomized trial focused on risk patients. “Risk” was defined either by age below 2 years or by involvement of at least one risk organ (RO; e.g. liver, spleen, hematopoietic system, or lung) irrespective of age. The continuation therapy included 6-MP in addition to the prednisolone/ vinblastine pulses for a total treatment duration of 6 months.


  • In a multivariable analysis, age at diagnosis <2 years has no prognostic significance and, therefore, the risk group can be reliably defined at diagnosis by the presence of RO involvement only.
  • Patients with RO involvement have a significant disease-related mortality and therapy intensification in this group is reasonable
  • Despite similar initial therapy, the overall treatment duration of 6 months in the LCH-II study resulted in a higher reactivation rate (44%) compared to 24% after 12-month treatment duration in the historical cohort of the DAL-HX studies


1. Gadner H, Grois N, Potschger U, Minkov M, Arico M, Braier J, et al. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood, 2008, 111(5):2556-62.

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