LCH-S-98

LCH-S-98 (1999-2004):

This salvage approach was designed as a non-randomized phase II study, aiming to evaluate the activity of cladribine (2-chlorodeoxyadenosine;  2-CdA) in patients with risk organ involvement (RO+), whose disease progressed after combination therapy with prednisolone, vinblastine and etoposide and in patients without risk organ involvement (RO-) with multiple disease reactivations (≥ 2 episodes).¹ 2-CdA was given as a 5-day course at a dose of 5 mg/m²/day, repeated every 3-4 weeks for 2-6 courses depending on response. Forty-six RO+ and 37 RO- patients were evaluable. 2-CdA as monotherapy was significantly more effective in RO- patients with a response in 62% compared to 22% in RO+ patients. Two-year predicted survival was 48% for RO+ and 97% for RO- patients. Thus, 2-CdA as a single drug was not sufficient to change the fate of the patients with most severe disease.

REFERENCES:

1. Weitzman S, Braier J, Donadieu J, Egeler RM, Grois N, Ladisch S, et al. 2'-Chlorodeoxyadenosine (2-CdA) as salvage therapy for Langerhans cell histiocytosis (LCH). results of the LCH-S-98 protocol of the histiocyte society. Pediatr Blood Cancer, 2009, 53(7):1271-6.