levoleucovorin and Histiocytosis--Langerhans-Cell

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.. 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).. 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.. 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.

Topics: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Bleomycin; Cyclophosphamide; Disease Progression; Doxorubicin; Female; Fluorodeoxyglucose F18; Follow-Up Studies; Histiocytosis, Langerhans-Cell; Humans; Leucovorin; Male; Methotrexate; Middle Aged; Positron-Emission Tomography; Prednisone; Remission Induction; Retrospective Studies; Vincristine; Young Adult

Topics: Adult; Age Factors; Age of Onset; Antineoplastic Combined Chemotherapy Protocols; Bleomycin; Child; Clinical Trials as Topic; Cyclophosphamide; Doxorubicin; Histiocytosis, Langerhans-Cell; Humans; Leucovorin; Methotrexate; Pediatrics; Prednisone; Retrospective Studies; Vincristine