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Human Herpes virus 8-negative primary effusion lymphoma with BCL6 rearrangement in a patient with idiopathic CD4 positive T-lymphocytopenia

¹ Department of Pathology
² Department of Internal Medicine, National National Hospital Organization Nagasaki Medical Center, Omura
³ Department of Hematology, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki
⁴ Department of Laboratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki
⁵ Department of Hematology, Sasebo Municipal General Hospital, Sasebo, Japan

Correspondence: Daisuke Niino, M.D., Ph.D., Department of Pathology, National Nagasaki Medical Center, 2-1001-1, Kubara, Omura, Nagasaki 856-8562, Japan. Phone: +81.957.523121. Fax: +81.957.540292. E-mail: niino522{at}yahoo.co.jp

Primary effusion lymphoma (PEL) was initially designated as a body-cavity-based lymphoma and recognized as a distinct clinical entity without a contiguous tumor mass. PEL was first reported in patients with acquired immunodeficiency syndrome (AIDS) and the distinctive feature of PEL originally reported as a B-cell neoplasm characterized by infection of the tumor cells by human herpes virus 8 (HHV-8).¹ However, there have recently been several reports of PEL in patients without human immunodeficiency virus (HIV) or HHV8 infection.^2–4

Key words: primary effusion lymphoma, Idiopathic CD4 positive T-lymphocytopenia, BCL6 rearrangement, Epstein-Barr virus, chemotherapy.