Annals of Oncology 15:1215-1221, 2004
© 2004 European Society for Medical Oncology
Anthracycline-based chemotherapy as primary treatment for intravascular lymphoma
Background: Optimal therapeutic management of intravascular lymphoma (IVL) lacks precise guidelines.Patients and methods: The clinico-pathological features of 38 HIV-negative patients with IVL were reviewed to define efficacy of chemotherapy in these malignancies. Clinical characteristics of 22 patients treated with chemotherapy and of 16 untreated patients were compared in order to understand better the impact and causes of potential patient selection.
Results: Median age was 70 years (range 34–90), with a male/female ratio of 0.9; 23 (61%) patients had Eastern Cooperative Oncology Group performance status (ECOG-PS) >1; 21 (55%) had systemic symptoms. Cutaneous lesions and anemia were significantly more common among patients treated with chemotherapy; central nervous system (CNS) and renal involvement were significantly more common among untreated patients. Chemotherapy was associated with a response rate of 59% and a 3-year overall survival of 33 ± 11%. Five of six patients with CNS involvement received chemotherapy: four of them died early; only one patient, treated with adriamycin, cyclophosphamide, vincristine, methotrexate, bleomycin and prednisolone (MACOP-B) followed by high-dose chemotherapy and autologous stem cell transplantation (ASCT), was alive at 19 months. High-dose chemotherapy supported by ASCT was indicated at diagnosis in another patient (43 years of age, stage I), who was alive at 71 months, and at relapse after cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) in two patients who died early after transplantation. PS 
1, disease limited to the skin, stage I, and use of chemotherapy were independently associated with better outcome.
Conclusions: Anthracycline-based chemotherapy is the standard treatment for IVL. However, survival is disappointing, with a relevant impact of diagnostic delay and lethal complications. More intensive combinations, containing drugs with higher CNS bioavailability, are needed in cases with brain involvement, and the role of high-dose chemotherapy supported by ASCT should be further investigated in younger patients with unfavorable features.
1 Department of Radiochemotherapy, San Raffaele H Scientific Institute, Milan, Italy; 2 Division of Pathology, Hospital Clínic, Barcelona, Spain; 3 Division of Hematology, Policlinico G B Rossi, Verona; 4 Divisions of Hematology, Ospedale Santa Maria, Reggio Emilia, Italy; 5 Australasian Leukaemia and Lymphoma Group, Richmond, Australia; 6 Dutch Cutaneous Lymphoma Group, Leiden, The Netherlands; 7 Department of Pathology, San Raffaele H Scientific Institute, Milan; 8 Division of Hematology, Spedali Civili di Brescia, Brescia, Italy; 9 Division of Hematology, Hospital Clínic, Barcelona, Spain; 10 Division of Dermatology, IRCCS Ospedale Maggiore, Milan, Italy; 11 Department of Oncology, University Hospital, Lund, Sweden; 12 Division of Hematology, Policlinico di Modena; 13 Division of Hematology, Ospedali Riuniti di Bergamo; 14 Division of Pathology, Policlinico G B Rossi, Verona; 15 Division of Pathology, Ospedale Santa Maria, Reggio Emilia, Italy; 16 Division of Pathology, Ist Oncologico Svizzera Italiana, Bellinzona, Switzerland; 17 Division of Pathology, Spedali Civili di Brescia, Brescia; 18 Division of Pathology, Ospedali Riuniti di Bergamo, Bergamo; 19 Division of Pathology, Ospedale Sacco, Milan; 20 Division of Pathology, Policlinico di Modena, Modena; 21 Division of Pathology, Ospedale Civile Maggiore Az. Ospedaliera, Verona, Italy; 22 Division of Medical Oncology, Ist Oncologico Svizzera Italiana, Bellinzona, Switzerland
* Correspondence to: Dr A. J. M. Ferreri, Department of Radiochemotherapy, San Raffaele H Scientific Institute, via Olgettina 60, 20132 Milan, Italy. Tel: +39-02-26437649; Fax: +39-02-26437603; Email: andres.ferreri{at}hsr.it
Key words: angiotropic lymphoma, chemotherapy, CHOP regimen, CNS lymphoma, cutaneous lymphoma, intravascular lymphomatosis
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