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.

MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: Experience on seven patients / 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. - In: ANNALS OF ONCOLOGY. - ISSN 0923-7534. - 21:6(2010), pp. 1173-1178. [10.1093/annonc/mdp455]

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

E. Derenzini;
2010

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.
chemotherapy; Langerhans cell histiocytosis; MACOP-B
Settore MED/15 - Malattie del Sangue
2010
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/724906
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