ABSTRACT
Intravascular large B-cell lymphoma (IVL), or ‘angiotropic lymphoma’, was earlier regarded, since the original description in 1959 by L. Pfleger and J. Tappeiner, as an endothelial neoplasia with vascular dissemination. Tissue biopsy is usually mandatory for the diagnosis of IVL; this is due to the lack of a pathognomonic diagnostic immunophenotypic marker for this lymphoma. Most importantly, blood vessel lumen, other than being merely a vehicle, is also a site of active replication for IVL cells; indeed, mitotic features are often easily recognizable within the lymphomatous population. Neuroimaging discloses central nervous system involvement only in half of patients with neurological symptoms. Patients with cutaneous variant of IVL survive significantly longer than others, independently of the International Prognostic Index and all the other prognostic variables investigated. Radiotherapy may be considered the exclusive treatment for IVL in elderly patients with single cutaneous lesions; outside this context, the role of radiotherapy remains to be determined.
