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


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.
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'. Electronic address: enrico.derenzini@libero.it.
E
E
Derenzini
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
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MP
Fina
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
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V
Stefoni
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
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C
Pellegrini
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
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F
Venturini
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
A
A
Broccoli
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
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L
Gandolfi
Unit of Haematopathology, Department of Haematology and Medical Oncology 'L. & A. Seràgnoli', University of Bologna.
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Pileri
Department of Nuclear Medicine, Sant'Orsola-Malpighi Hospital, Bologna, Italy.
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Fanti
Department of Nuclear Medicine, Sant'Orsola-Malpighi Hospital, Bologna, Italy.
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E
Lopci
Department of Nuclear Medicine, Sant'Orsola-Malpighi Hospital, Bologna, Italy.
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P
Castellucci
Unit of Haematopathology, Department of Haematology and Medical Oncology 'L. & A. Seràgnoli', University of Bologna.
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C
Agostinelli
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
M
M
Baccarani
Department of Haematology and Medical Oncology 'L. & A. Seràgnoli'.
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PL
Zinzani
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001761:Bleomycin / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D019788:Fluorodeoxyglucose F18; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging / Q000188:drug therapy*; D006801:Humans; D002955:Leucovorin / Q000627:therapeutic use; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D049268:Positron-Emission Tomography; D011241:Prednisone / Q000627:therapeutic use; D012074:Remission Induction; D012189:Retrospective Studies; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
D019788:Fluorodeoxyglucose F18; D001761:Bleomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D002955:Leucovorin; D011241:Prednisone; D008727:Methotrexate