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Primary Effusion Lymphoma : Introduction

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Introduction: Primary effusion lymphoma (PEL) is a distinct subtype of lymphoma that presents as malignant effusion involving pleural, pericardial, or peritoneal cavities. It is associated with human herpesvirus 8 (HHV8) also known as Kaposi Sarcoma-associated Herpesvirus (KSHV). Most patients are adult males with AIDS and have severe immunodeficiency; however, it can also occur in females and in other settings, such as following organ transplantation.

PEL, by definition, is body-cavity based and presents without detectable tumor masses. Rare patients develop secondary tumor nodules in adjacent structures. In addition, there are rare lymphomas resembling PEL that are extracavitary. They develop as solid masses in the lymph nodes or gastrointestinal tract and are not associated with an effusion. They are called extracavitary PEL.

This chest CT shows bilateral pleural effusion in a HIV+ male with primary effusion lymphoma.

Image courtesy of: Genevieve Crane, MD, PhD.

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