ETOPOSIDE AS THE BASIC AND INTERFERON-a AS THE MAINTENANCE THERAPY FOR LANGERHANS CELL HISTIOCYTOSIS: A RTC

Abstract

The treatment of patients who suffer from a disseminated form of Langerhans cell histiocytosis (LCH) is still controversial. So far, few larger randomized studies have been performed. The authors present 3 patients with a disseminated form of LCH--4 months, 9 months, and 2 years old, respectively. The lesional Langerhans cells in each patient showed positive immunohistochemical reaction to S-100 protein and the presence of Birbeck granules was confirmed by electron microscopy. All the patients were treated with etoposide (VP-16), 200 mg/m2 for 3 consecutive days, with 15 cycles at intervals of 3 weeks between each cycle, followed by maintenance therapy with IFN-alpha. All 3 patients reached complete stabile remission. The patients were young, at high risk, with multiple-organ involvement of LCH, and two of them had obvious signs of organ dysfunction at presentation, suggesting a poor prognosis. All remain disease-free several years after therapy. The results suggest that INF-alpha may prevent recurrences in high-risk patients.

Association of both Langerhans cell histiocytosis and Erdheim-Chester disease linked to the BRAFV600E mutation

“…IFN-a was consequently the most frequently used therapy because it has also been proposed to treat LCH. [42][43][44][45] However, the efficacy of IFN-a was lower than what has been reported previously for isolated-ECD patients. [46][47][48] These differences cannot be explained by the clinical spectrum of mixed histiocytosis, the dose of IFN-a, or the duration of the follow-up, which were equivalent in our series and in the previous series of patients with isolated ECD.…”

Strahlentherapie ossärer Manifestationen der Langerhans-Zell-Histiozytose beim Erwachsenen. Review und Vorstellung eines internationalen Registers

“…CT represents the treatment of choice to permit systemic disease control [4, 5, 9, 13, 17-19, 21, 22, 25, 30, 31, 39, 42, 44, 50, 54]. Immunosuppressive drugs can support the systemic effect especially in children [12,38]. Often, RT is required for the control of imminent local symptoms (see above).…”

Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell–macrophage lineage

“…Some sporadic patients have been treated with agents like cyclosporine‐A (17), thalidomide (18), and interferon‐alpha (IFN‐α) (19), all drugs with significant activity in low‐risk LCH. These results, however, are based on a few patients, and non‐published discussions through the grape‐vine indicate no long‐lasting results.…”