An unusual case of Primary Effusion Lymphoma with aberrant T-cell phenotype in a HIV-negative, HBV-positive, cirrhotic patient, and review of the literature

Abstract

<jats:p>Primary effusion lymphoma (PEL) is an unusual, human herpes virus-8 (HHV-8)–associated type of lymphoma, presenting as lymphomatous effusion in body cavities, without a detectable tumor mass. It primarily affects human immunodeficiency virus (HIV)-infected patients, but has also been described in other immunocompromised individuals. Although PEL is a B-cell lymphoma, the neoplastic cells are usually of the ‘null’ phenotype by immunocytochemistry. This report describes a case of PEL with T-cell phenotype in a HIV-negative patient and reviews all the relevant cases published until now. Our patient suffered from cirrhosis associated with Hepatitis B virus (HBV) infection and presented with a large ascitic effusion, in the absence of peripheral lymphadenopathy or solid mass within either the abdomen or the thorax. Paracentesis disclosed large lymphoma cells with anaplastic features consisting of moderate cytoplasm and single or occasionally multiple irregular nuclei with single or multiple prominent nucleoli. Immunocytochemically, these cells were negative for both CD3 and CD20, but showed a positive reaction for T-cell markers CD43 and CD45RO (VCHL-1). Furthermore, the neoplastic cells revealed strong positivity for EMA and CD30, but they lacked expression of ALK-1, TIA-1, and Perforin. The immune status for both HHV-8 and Epstein-Barr virus (EBV) was evaluated and showed positive immunostaining only for the former. The combination of the immunohistochemistry results with the existence of a clonal rearrangement in the immunoglobulin heavy chain gene (identified by PCR), were compatible with the diagnosis of PEL. The presence of T-cell markers was consistent with the diagnosis of PEL with an aberrant T-cell phenotype.</jats:p>

Primary effusion lymphoma with aberrant T‐cell phenotype in an iatrogenically immunosuppressed renal transplant male: Cytologic diagnosis in peritoneal fluid

“…Accordingly, after completion of the relative tests, our case has shown the presence of HHV‐8 and the absence of EBV infection. Previous molecular studies have approved the occurrence of immunoglobulin gene rearrangements (this is also true in our case) and somatic hypermutation in PEL cells, indicating that the cell of origin is a post‐germinal center B‐cell . The occurrence of a T‐cell immunophenotype in PEL is a relatively rare event and only a few cases have been published until now .…”

“…Immunocytochemically, both are positive for CD30 and EMA. Furthermore, ALCL demonstrates a T‐cell immunophenotype . With regard to all the arguments raised herewith, while keeping in mind that our case also showed a T‐cell profile, it was difficult to exclude an ALCL.…”

“…Our case was also positive for HHV‐8 by in situ hybridism, a finding that along with the particular cytomorphological and immunocytochemical findings, confirmed that the lymphomatous effusion was a case of PEL. The standard assay to detect HHV‐8 infection in tissues is the immunocytochemical staining for Latent Nuclear Antigen‐1 (LANA‐1), or in situ hybridism (in our case) whereas, EBV infection is most reliably identified by in situ hybridization for EBV‐RNA, as immunocytochemical staining for EBV latent membrane protein (LMP‐1) is almost always negative . Accordingly, after completion of the relative tests, our case has shown the presence of HHV‐8 and the absence of EBV infection.…”

“…With regard to all the arguments raised herewith, while keeping in mind that our case also showed a T‐cell profile, it was difficult to exclude an ALCL. However, the strong positivity for HHV‐8 established the diagnosis of PEL as the most possible . As far as DLBCL and in particular the immunoblastic variant, confusion may arise, as this may also show morphological features similar to PEL.…”

Announcement of first time Cytojournal impact factor for 2012 coincides with Cytojournal decade celebration (2004-2013)

“…The 2012 CytoJournal articles cited during 2013 will contribute to the forthcoming IF for 2013. [6768697071727374757677787980818283848586878889909192]…”