Biomodulatory therapy induces durable remissions in multi-system Langerhans cell histiocytosis. Issue 12 (15th October 2022)

• Record Type: Journal Article
• Authors: Harrer, Dennis C.; Jakob, Marcus; Vogelhuber, Martin; Lüke, Florian; Utpatel, Kirsten; Corbacioglu, Selim; Herr, Wolfgang; Reichle, Albrecht; Heudobler, Daniel
• Is Part Of: Leukemia & lymphoma. Volume 63:Issue 12(2022)
• Page Start: 2858
• Page End: 2868
• Publication Date: 2022-10-15
• Subjects: Langerhans-cell-histiocytosis -- biomodulation -- anakoinosis -- metronomic chemotherapy -- anti-inflammation; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2022.2095627
• Languages: English
• ISSNs: 1042-8194

Abstract

Abstract: Langerhans cell histiocytosis (LCH) is rare hematological neoplasia originating from the aberrant proliferation of CD207-positive dendritic cells. Refractory multi-system LCH is difficult to treat necessitating the continuous development of different salvage therapies. At our medical center, eleven patients (age 11 months to 77 years) with multi-system LCH were treated on a compassionate use basis with metronomic biomodulation therapy (MBT) involving the daily oral application of low-dose trofosfamide, etoricoxib, pioglitazone and low-dose dexamethasone. Overall, four patients including two heavily pretreated pediatric patients achieved ongoing complete remission. Moreover, partial disease remission was observed in three patients, and four patients attained stable disease. MBT demonstrated high activity against multi-system LCH even in patients, refractory to multiple systemic chemotherapies. Further confirmation of efficacy should be systematically evaluated in prospective trials.