Disseminated Langerhans cell histocytosis in children: treatment outcome.

Abstract

Langerhans cell histiocytosis is an interesting disorder with a variety of presentations and variable outcomes. This study evaluates response to treatment, recurrence, and survival in disseminated Langerhans cell histiocytosis treated at Regional Cancer Centre, Trivandrum, India from 1983 through 1994. Thirty-five patients with disseminated Langerhans cell histiocytosis were seen. Six had group A disease, 21 had group B disease, and eight had group C disease. In group A, five of six patients are disease free at a median follow-up of 48 months. Two had recurrence after initial treatment, which was salvaged. In group B, 13 of 15 patients had complete response after chemotherapy, nine of whom experienced recurrence later. Three of five patients who received irradiation alone experienced recurrence. One died of progressive disease. Two patients were lost to follow-up. Seventeen of 20 are alive with median follow-up of 67 months. In group C, one of eight patients are alive after multiple recurrences. Of the surviving patients, 29% had significant sequelae. In summary, group A and B patients do well with treatment, and most of the recurrences can be salvaged. A significant proportion of patients have sequelae. Newer aggressive protocols must be developed for treating group C patients. Measures to prevent sequelae must also be developed.