LCH-III: therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis
Twelve months of vinblastine and prednisone, rather than six, meant fewer children's Langerhans cell histiocytosis came back, and mortality in the highest-risk children fell to a fraction of what it had been.
Overview
International randomised trial of the Histiocyte Society in children with multisystem Langerhans cell histiocytosis: patients with risk-organ involvement were randomised to vinblastine and prednisone with or without methotrexate, and those without risk-organ involvement to six or twelve months of treatment.
Methotrexate added no benefit, while twelve months of therapy roughly halved the five-year reactivation rate compared with six months (about 37 versus 54 percent). Mortality in risk-organ-positive patients fell to about 15 percent with early switching of non-responders to salvage. Twelve months of vinblastine and prednisone became the international standard.
- Twelve months of vinblastine and prednisone reduced five-year reactivation to about 37 percent from about 54 percent with six months.
- Adding methotrexate did not improve outcomes in risk-organ-positive patients.
Children with multisystem LCH receive a year of vinblastine and prednisone, and those in whom the disease does not respond quickly are switched early to salvage therapy.
- Reactivation remains common even after twelve months, and late effects such as neurodegeneration are not prevented.
- Adults were not studied; adult regimens are extrapolated.
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