MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients.

Abstract

BACKGROUND:

Adult Langerhans cell histiocytosis (LCH) is a rare disease. The combination of vinblastine and prednisone, given in a 6-month course, is the standard of care but prospective randomized trials are lacking.

PATIENTS AND METHODS:

We report our monocentric experience in the treatment of seven adult patients with multisystem (MS) LCH (n = 3) or single-system multifocal (SS-m) LCH (n = 4) with the short-course intensive chemotherapy regimen methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone and bleomicin (MACOP-B).

RESULTS:

The overall response rate was 100% [five complete response (CR), two partial response (PR)]. After a median follow-up of 6.5 years, four patients are in first continuous CR and three patients relapsed after 5, 8 and 62 months, respectively. Four patients were evaluated with positron emission tomography (PET) scan: all three PET-negative patients at the end of treatment had a long-lasting response with only one patient relapsing after 5 years. PET scan detected additional bone lesions at diagnosis in two of four patients, changing the treatment program in one of them.

CONCLUSIONS:

MACOP-B regimen seems to be very active in the treatment of adult MS or SS-m LCH, with long-lasting responses in five of seven patients. PET scan merits further evaluation in the initial staging and in the evaluation of the response to chemotherapy.

• Authors: E Derenzini, M P Fina, V Stefoni, C Pellegrini, F Venturini, A Broccoli, L Gandolfi, S Pileri, S Fanti, E Lopci, P Castellucci, C Agostinelli, M Baccarani, P L Zinzani
• Journal: Annals of oncology : official journal of the European Society for Medical Oncology (Ann Oncol) Vol. 21 Issue 6 Pg. 1173-1178 (Jun 2010) ISSN: 1569-8041 [Electronic] England
• PMID: 19861578 (Publication Type: Evaluation Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Fluorodeoxyglucose F18
• Bleomycin
• Vincristine
• Doxorubicin
• Cyclophosphamide
• Leucovorin
• Prednisone
• Methotrexate

Topics

• Adolescent
• Adult
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Bleomycin (therapeutic use)
• Cyclophosphamide (therapeutic use)
• Disease Progression
• Doxorubicin (therapeutic use)
• Female
• Fluorodeoxyglucose F18
• Follow-Up Studies
• Histiocytosis, Langerhans-Cell (diagnostic imaging, drug therapy)
• Humans
• Leucovorin (therapeutic use)
• Male
• Methotrexate (therapeutic use)
• Middle Aged
• Positron-Emission Tomography
• Prednisone (therapeutic use)
• Remission Induction
• Retrospective Studies
• Vincristine (therapeutic use)
• Young Adult