Langerhans-cell histiocytosis in children

Abstract

Histiocytosis from Langerhans cells is a new term for a group of diseases formerly called histiocytosis X. In The Faculty Hospital Motol the authors treated between July 1974 and December 1980 84 children with this disease. Twenty one suffered from the malignant form (formerly Letterer-Siwe and Hand-Schüller-Christian disease) and in 63 patients the diagnosis of eosinophil granuloma was established. In 21 children with unequivocally malignant disease the authors started chemotherapy immediately after establishment of the diagnosis and in six they indicated in addition radiotherapy. Of these 21 children 16 are in complete remission and 5 children died from progression of the disease during treatment. In the group of 63 children with eosinophil granuloma in 44 only excochleation of the focus was performed. Chemotherapy was administered to 12 children, incl. 5 where it was combined with radiotherapy. A relapse of the disease was recorded in 8 children. At present all patients suffering from the disease are in complete remission.