Intravascular Lymphoma: The Oncologist's “Great Imitator”

Abstract

Abstract Learning Objectives After completing this course, the reader will be able to: Describe the clinicopathologic features of IVL that distinguish it from the other large-cell lymphomas.Discuss the diagnostic tests helpful in evaluating a case of suspected IVL.Explain the rationale for treatment approaches in patients with IVL, including the role of hematopoietic stem cell transplantation. Access and take the CME test online and receive 1 AMA PRA category 1 credit at CME.TheOncologist.com Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large B-cell lymphoma with a distinct presentation. Anatomically the disease is characterized by the proliferation of clonal lymphocytes within small vessels with relative sparing of the surrounding tissue. The clinical symptoms of the disease are dependent on the specific organ involvement, which most often includes the central nervous system and skin. Because of the various modes of presentation and the rarity of IVL, the diagnosis is often made postmortem. The diagnosis is almost exclusively made by surgical biopsy of a suspected site of involvement. Advances in imaging and immunohistochemistry have led to increasing antemortem diagnosis of this lymphoma. Although some patients with this disease may be curable with aggressive therapy, further research into novel treatment strategies is needed to improve outcome. Some potential insights into future therapies may be drawn from the small amount of basic science literature relevant to this entity. This review provides a concise, up-to-date summary of IVL.