Originally published as JCO Early Release 10.1200/JCO.2006.08.2313 on June 18 2007

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Definition, Diagnosis, and Management of Intravascular Large B-Cell Lymphoma: Proposals and Perspectives From an International Consensus Meeting

Maurilio Ponzoni, Andrés J.M. Ferreri, Elías Campo, Fabio Facchetti, Luca Mazzucchelli, Tadashi Yoshino, Takuhei Murase, Stefano A. Pileri, Claudio Doglioni, Emanuele Zucca, Franco Cavalli, Shigeo Nakamura

From the Unit of Lymphoma Malignancies, Pathology and Oncology Units, Department of Oncology, San Raffaele Scientific Institute, Milan; Department of Pathology I, University of Brescia School of Medicine, Spedali Civili of Brescia, Brescia; Hematopathology, Bologna University School of Medicine, Bologna, Italy; Laboratory of Pathology, Hospital Clinic, University of Barcelona, Barcelona, Spain; Istituto di Patologia, Locarno; Istituto Oncologico della Svizzera Italiana, Ospedale S Giovanni, Bellinzona, Switzerland; Department of Pathology, Okayama University, Okayama; Department of Internal Medicine, Nishio Municipal Hospital, Aichi; and the Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Nagoya, Japan

Address reprint requests to Maurilio Ponzoni, MD, Pathology Unit, San Raffaele Scientific Institute, Via Olgettina 60, 20132 Milano, Italy; e-mail: ponzoni.maurilio{at}hsr.it

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of diffuse LBCL characterized by preferential intravascular growth of malignant lymphocytes, aggressive behavior, and an often fatal course. IVLBCL usually affects elderly patients with poor performance status, elevated lactic dehydrogenase serum levels, anemia, and B symptoms. It displays some differences in clinical presentation among diverse geographical areas, mostly between patients diagnosed in Western countries and Japan. In addition, data from the literature suggest that pathologic diagnostic criteria as well as clinical features of this disease may be broader than described in current classification scheme(s). Under the sponsorship of the International Extranodal Lymphoma Study Group, clinicians and pathologists with interest in IVLBCL, coming from Western and Eastern countries, joined to reach a consensus on defining features as well as to focus on the most urgent unresolved issues in IVLBCL. To this end, a representative group of IVLBCL patients coming from both the aforementioned geographical areas were collectively analyzed. Additional features of IVLBCL were proposed both under clinical and pathologic stand points. At the meeting, it emerged that IVLBCL may have additional histopathologic/cytologic definition criteria with respect to those currently recommended, some clinical features are not randomly distributed worldwide, recent therapeutic approaches, such as anti-CD20–containing regimens, may improve outcome, and kidney, spleen, and liver involvement may show peculiar histopathologic features. Finally, a provisional practical diagnostic approach to hemophagocytosis-associated patients and a proposal for the most useful criteria in the settings of differential diagnosis are included.

published online ahead of print at www.jco.org on June 18, 2007.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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