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

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)1173-1178
Number of pages6
JournalAnnals of Oncology
Issue number6
Publication statusPublished - Oct 27 2009


  • Chemotherapy
  • Langerhans cell histiocytosis

ASJC Scopus subject areas

  • Oncology
  • Hematology
  • Medicine(all)


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