TY - JOUR
T1 - MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis
T2 - Experience on seven patients
AU - Derenzini, E.
AU - Fina, M. P.
AU - Stefoni, V.
AU - Pellegrini, C.
AU - Venturini, F.
AU - Broccoli, A.
AU - Gandolfi, L.
AU - Pileri, S.
AU - Fanti, S.
AU - Lopci, E.
AU - Castellucci, P.
AU - Agostinelli, C.
AU - Baccarani, M.
AU - Zinzani, P. L.
PY - 2009/10/27
Y1 - 2009/10/27
N2 - 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 longlasting responses in five of seven patients. PET scan merits further evaluation in the initial staging and in the evaluation of the response to chemotherapy.
AB - 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 longlasting responses in five of seven patients. PET scan merits further evaluation in the initial staging and in the evaluation of the response to chemotherapy.
KW - Chemotherapy
KW - Langerhans cell histiocytosis
KW - MACOP-B
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U2 - 10.1093/annonc/mdp455
DO - 10.1093/annonc/mdp455
M3 - Article
C2 - 19861578
AN - SCOPUS:77953986335
VL - 21
SP - 1173
EP - 1178
JO - Annals of Oncology
JF - Annals of Oncology
SN - 0923-7534
IS - 6
ER -