Current understanding and future prospects for intravascular large B-cell lymphoma

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare distinct disease entity of the extranodal large B-cell lymphoma type, characterized by the selective growth of tumor cells in the lumina of small vessels of various organs. Lack of remarkable lymphadenopathy and non-specific clinical abnormalities including fever of unknown origin and lactate dehydrogenase elevation generally make timely and accurate diagnosis difficult. Recent diagnostic advances in the detection of this disease using FDG-PET/CT and random skin biopsies are expected to increase the diagnostic yield. Regarding the therapeutic aspects of this disease, improvement of clinical outcomes by the application of anti-CD20 monoclonal antibody, rituximab, and a high risk of CNS recurrence have been indicated. Thus, a prospective phase II trial of immunochemotherapy combined with CNS prophylaxis is now ongoing. Previously, the difficulty of obtaining sufficient tumor samples hampered biological investigations but the novel technique developing xenograft models is opening the door to uncovering the underlying mechanisms by focusing on the fundamental biological question: "Why do tumor cells become lodged in the lumina of vessels?" This review describes the current understanding of research on IVLBCL and discusses the future prospects for this disease entity.