Intravascular large B-cell lymphoma (also termed angio-tropic lymphoma) is a malignant proliferation of large B lymphocytes within blood vessels. This disease was formerly classified as a vascular neoplasm (malignant angioendothe-liomatosis) . In rare cases, the skin may be the only affected site, although more often it is disseminated, with involvement of the central nervous system being common. Most cases of cutaneous intravascular lymphoma show a B-cell phenotype but a T-cell variant of the disease has been reported (see Chapter 8). Intravascular large B-cell lymphoma is included as a provisional entity in the European Organization for Research and Treatment of Cancer (EORTC) classification and as a distinct entity in the World Health Organization (WHO) classification [2,3].
The reason(s) why neoplastic cells in intravascular large B-cell lymphoma are confined within the vessels is unclear. The absence of molecules crucial for adhesion of lymphocytes to endothelial cells and migration out of the vessels (CD29, CD54) has been observed in some cases, leading to the hypothesis that neoplastic lymphocytes in intravascular large B-cell lymphoma are unable to escape outside of the vessel walls .
Intravascular large B-cell lymphoma may arise in patients with pre-existing cutaneous or, more often, nodal large B-cell lymphoma, probably representing in these cases recurrence of the original disease [5,6].
Was this article helpful?