Participants and collaborating centres according to the number of patients entered (n): A. J. M. Ferreri, S. Govi, S. Dell'Oro and M. Ponzoni, San Raffaele H Scientific Institute, Milan, Italy (six); A. López-Guillermo and E. Campo, Hospital Clínic, Barcelona, Spain (four); J. F. Seymour, Australasian Leukaemia and Lymphoma Group (three); R. Willemze, Dutch Cutaneous Lymphoma Group, the Netherlands (three); F. Illariucci and S. Asioli, Ospedale Santa Maria, Reggio Emilia, Italy (three); A. Ambrosetti and M. Lestani, Policlinico G B Rossi, Verona, Italy (three); S. Grisanti, E. Pedrinis and F. Cavalli, Istituto Oncologico della Svizzera Italiana, Switzerland (two); G. Rossi, M. Ungari and F. Facchetti, Spedali Civili di Brescia, Italy (two); E. Berti, IRCCS Ospedale Maggiore, Milan, Italy (one); M. A. Pavlovsky, Fundaleu, Buenos Aires, Argentina (one); M. Martelli, Università La Sapienza, Rome, Italy (one); M. L. Geerts, University Hospital, Gent, Belgium (one); M. Eriksson, University Hospital, Lund, Sweden (one); M. Federico and T. Artusi, Policlinico di Modena, Italy (one); A. Candoni, Policlinico Universitario Udine, Italy (one); M. A. Piris, Centro Nacional de Investigaciones Oncológicas, Madrid, Spain (one); S. Cortelazzo and T. Motta, Ospedali Riuniti di Bergamo, Italy (one); A. De Renzo, Università Federico II, Napoli, Italy (one); M. Milani, Azienda Ospedaliera di Lecco, Italy (one); R. Rossi, Ospedale Sacco, Milan, Italy (one); S. Ascani and S. Pileri, Ospedale Sant'Orsola, Bologna, Italy; C. Patriarca, Ospedale Vizzolo Predabissi, Melegnano, Italy.
Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the ‘cutaneous variant’1
Article first published online: 1 OCT 2004
British Journal of Haematology
Volume 127, Issue 2, pages 173–183, October-II 2004
How to Cite
Ferreri, A. J. M., Campo, E., Seymour, J. F., Willemze, R., Ilariucci, F., Ambrosetti, A., Zucca, E., Rossi, G., López-Guillermo, A., Pavlovsky, M. A., Geerts, M.-L., Candoni, A., Lestani, M., Asioli, S., Milani, M., Piris, M. A., Pileri, S., Facchetti, F., Cavalli, F., Ponzoni, M. and the International Extranodal Lymphoma Study Group (IELSG) (2004), Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the ‘cutaneous variant’. British Journal of Haematology, 127: 173–183. doi: 10.1111/j.1365-2141.2004.05177.x
- Issue published online: 1 OCT 2004
- Article first published online: 1 OCT 2004
- Received 28 June 2004; accepted for publication 8 August 2004
- extranodal lymphoma;
- angiotropic lymphoma;
- intravascular lymphoma;
- cutaneous lymphoma;
- central nervous system lymphomas
Despite its recognition as a distinct, extremely rare entity, no large studies of intravascular lymphoma (IVL) have been reported. The clinico-pathological characteristics of 38 human immunodeficiency virus-negative patients with IVL diagnosed in Western countries were reviewed to better delineate clinical presentation, clinical variants, natural history and optimal therapy. The IVL is an aggressive and usually disseminated disease (Ann Arbor stage IV in 68% of cases) that predominantly affects elderly patients (median age 70 years, range: 34–90; male:female ratio 0·9), resulting in poor Eastern Cooperative Oncology Group Performance Status (ECOG-PS >1 in 61%), B symptoms (55%), anaemia (63%) and high serum lactate dehydrogenase level (86%). The brain and skin are the most common sites of disease. In contrast to previous reports, hepatosplenic involvement (26%) and bone marrow infiltration (32%) were found to be common features in IVL, while nodal disease was confirmed as rare (11% of cases). Patients with disease limited to the skin (‘cutaneous variant’; 26% of cases) were invariably females with a normal platelet count, and exhibited a significantly better outcome than the remaining patients, which deserves further investigation. Overall survival was usually poor; however, the early use of intensive therapies could improve outcome in young patients with unfavourable features. ECOG-PS >1, ‘cutaneous variant’, stage I and chemotherapy use were independently associated with improved survival.