T Cell Primary Effusion Lymphoma in an HIV-Negative Man with Liver Cirrhosis

Abstract

Patient: Male, 73-year-old

Final Diagnosis: T cell lymphoma

Symptoms: Dyspnea

Medication: —

Clinical Procedure: Thoracentesis

Specialty: Oncology

Objective:

Rare disease

Background:

Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin lymphoma (NHL) that is responsible for 1% of all lymphomas not related to human immunodeficiency virus (HIV). PEL is characterized by human herpesvirus-8 (HHV-8) positivity in the absence of overt tumor burden that does not exhibit typical B cell or T cell immunophenotype characteristics. The exact mechanism of development is unknown, but it is hypothesized to develop from post-germinal B cell origin. Although it is most common in HIV patients, other immuno-compromising comorbidities can be seen in conjunction with PEL, including liver cirrhosis.

Case Report:

We present the case of a 73-year-old HIV-seronegative man with alcohol-induced liver cirrhosis who was found to have T cell PEL of the pleural space diagnosed by thoracentesis.

Conclusions:

Little is known regarding oncogenesis of T cell PEL, and few studies exist regarding appropriate treatment regimens for PEL as a whole, prompting need for further investigation and discussion to improve survival rates. Even in the absence of active HIV infection, PEL should be considered as a potential cause of pleural effusion in cirrhotic patients in order to prompt earlier treatment for the best chance of survival.