LCH-II

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.

MAIN FINDINGS OF LCH-II:

• 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

REFERENCES:

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.