Intravascular lymphoma: Clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'

Andrés J M Ferreri, Elías Campo, John F. Seymour, Rein Willemze, Fiorella Ilariucci, Achille Ambrosetti, Emanuele Zucca, Giuseppe Rossi, Armando López-Guillermo, Miguel A. Pavlovsky, Marie Louise Geerts, Anna Candoni, Maurizio Lestani, Silvia Asioli, Mario Milani, Miguel A. Piris, Stefano Pileri, Fabio Facchetti, Franco Cavalli, Maurilio Ponzoni

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)173-183
Number of pages11
JournalBritish Journal of Haematology
Volume127
Issue number2
DOIs
Publication statusPublished - Oct 2004

Keywords

  • Angiotropic lymphoma
  • Central nervous system lymphomas
  • Cutaneous lymphoma
  • Extranodal lymphoma
  • Intravascular lymphoma

ASJC Scopus subject areas

  • Hematology

Fingerprint Dive into the research topics of 'Intravascular lymphoma: Clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant''. Together they form a unique fingerprint.

  • Cite this

    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. (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(2), 173-183. https://doi.org/10.1111/j.1365-2141.2004.05177.x