High efficacy of the MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis, a 20 year experience.

Abstract

BACKGROUND:

Adult Langerhans cell histiocytosis (LCH) is an orphan disease. Chemotherapy is usually reserved to patients presenting with single system multifocal (SS-m) or multisystem (MS) disease but due to the lack of randomized studies no standard first line therapy has been defined yet. Pediatric regimens based on the vinblastine/prednisone backbone are not well tolerated in adults and probably less effective. We previously demonstrated high efficacy of the dose dense polichemotherapy regimen MACOP-B in 7 adult patients with SS-m or MS-LCH, in terms of high response rate and durable responses. Here we report an update of these data with the purpose of evaluating the long term efficacy of MACOP-B in adult LCH.

METHODS:

Clinical data of all adult LCH patients (n = 17) diagnosed and treated at our Institution during the past 20-year period were retrospectively reviewed.

RESULTS:

A total of 11 patients (6 with SS-m and 5 with MS-LCH) were treated with MACOP-B from 1995 to 2014. The overall response rate was confirmed to be 100 %, with a complete response of 73 % and a partial response rate of 27 %. Overall progression free survival was 64 %, and disease free survival after achievement of initial CR was 87 %. Overall survival rate was 82 % after 6.7 years of median follow-up.

CONCLUSIONS:

These data confirm high activity of MACOP-B in adult LCH, indicating that a substantial fraction of patients achieve long lasting responses and can be cured with this therapeutic approach.

• Authors: Enrico Derenzini, Vittorio Stefoni, Cinzia Pellegrini, Letizia Gandolfi, Alessandro Broccoli, Beatrice Casadei, Federica Quirini, Lisa Argnani, Lorenzo Tonialini, Pier Luigi Zinzani
• Journal: BMC cancer (BMC Cancer) Vol. 15 Pg. 879 (Nov 09 2015) ISSN: 1471-2407 [Electronic] England
• PMID: 26552668 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Bleomycin
• Vincristine
• Doxorubicin
• Cyclophosphamide
• Prednisolone
• Methotrexate

Topics

• Adolescent
• Adult
• Antineoplastic Combined Chemotherapy Protocols (administration & dosage)
• Bleomycin (administration & dosage)
• Cyclophosphamide (administration & dosage)
• Disease-Free Survival
• Doxorubicin (administration & dosage)
• Female
• Follow-Up Studies
• Histiocytosis, Langerhans-Cell (drug therapy, epidemiology, pathology)
• Humans
• Male
• Methotrexate (administration & dosage)
• Middle Aged
• Prednisolone (administration & dosage)
• Remission Induction
• Vincristine (administration & dosage)