Langerhans cell histiocytosis (LCH) is a rare disorder described as three different entities including eosinophilic granuloma
of bone, the Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. LCH is currently classified into single
system LCH, and multisystem LCH. Patients with single system LCH have an excellent prognosis, and are mostly
treated with local therapy. Multisystem LCH is subdivided into low risk and high risk groups. A 6-week course of
PRED/VBL is recommended for all patients with MS-LCH. Further therapy depends on the response to the initial
course, and risk group of the patient.