Biomodulatory therapy induces durable remissions in multi-system Langerhans cell histiocytosis

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.

Keywords:

• Langerhans-cell-histiocytosis
• biomodulation
• anakoinosis
• metronomic chemotherapy
• anti-inflammation

Ethics approval statement

The study was approved by the Ethics Committee of the University Regensburg (ethics statement No.: 18-1049-104).

Patient consent statement

Written informed consent for participation and publication was obtained from all patients.

Author contributions

MJ, MV, SC, WH, AR and DH treated the patients. DCH, MJ, FL, SC, AR and DH analyzed the data. DCH, FL, AR and DH wrote the manuscript. All authors revised the manuscript critically, approved the final manuscript, and agreed to be accountable for all aspects of the manuscript.

Disclosure statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Data availability statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.