Challenging differential diagnosis, Unusual or unexpected effect of treatment, Rare disease, Clinical situation which can not be reproduced for ethical reasons
Jacqueline Kropf, Mina Gerges, Ariel Perez Perez, Austin Ellis, Mevin Mathew, Kwabena Ayesu, Li Ge, Steve J. Carlan
Department of Internal Medicine, Orlando Regional Healthcare, Orlando, FL, USA
Am J Case Rep 2020; 21:e919032
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 immunocompromising 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.
Keywords: Liver Cirrhosis, Alcoholic, Lymphoma, Primary Effusion, Pleural Effusion, Malignant