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Intravascular large cell lymphoma

Authors
Arnold S Freedman, MD
Jon C Aster, MD
Almut Böer-Auer, MD
Section Editors
Richard A Larson, MD
John A Zic, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Intravascular large cell lymphoma (ILCL) is a rare subtype of large cell lymphoma that is characterized by the proliferation of lymphoma cells within the lumina of small blood vessels, particularly capillaries and post-capillary venules, without an obvious extravascular tumor mass or detectable circulating lymphoma cells in the peripheral blood.

This type of lymphoma has also been described as intravascular lymphomatosis, angiotropic large cell lymphoma, and malignant angioendotheliomatosis. The clinical presentation is varied and often includes symptoms related to organ dysfunction caused by occlusion of blood vessels. Particularly in the past, the diagnosis was usually only recognized at autopsy. In recent years, the heightened awareness of ILCL has resulted in more patients being diagnosed during life, thereby allowing for treatment.

Most information on ILCL comes from case reports and small case series of patients. In addition, an expert panel of pathologists and clinicians, convened under the sponsorship of the International Extranodal Lymphoma Study Group, has proposed practical guidelines [1].

The clinical presentation, diagnosis, and treatment of intravascular large cell lymphoma will be discussed here. Other types of large cell lymphoma are presented separately. (See "Epidemiology, clinical manifestations, pathologic features, and diagnosis of diffuse large B cell lymphoma" and "Initial treatment of advanced stage diffuse large B cell lymphoma".)

EPIDEMIOLOGY

ILCL is a rare subtype of large cell lymphoma; the true incidence is unknown. Median age at diagnosis is in the sixth to seventh decades; there is no sex predilection [2-4].

             

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Literature review current through: Nov 2016. | This topic last updated: Thu Dec 17 00:00:00 GMT+00:00 2015.
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