Primary Effusion Lymphoma: A Clinicopathologic Perspective

Abstract

Primary effusion lymphoma (PEL) is a rare, aggressive B-cell lymphoma that usually localizes to serous body cavities to subsequently form effusions in the absence of a discrete mass. Although some tumors can develop in extracavitary locations, the areas most often affected include the peritoneum, pleural space, and the pericardium. PEL is associated with the presence of human herpesvirus 8 (HHV8), also called the Kaposi sarcoma-associated herpesvirus (KSHV), with some variability in transformation potential suggested by frequent coinfection with the Epstein-Barr virus (EBV) (~80%), although the nature of the oncogenesis is unclear. Most patients suffering with this disease are to some degree immunocompromised (e.g., Human immunodeficiency virus (HIV) infection or post-solid organ transplantation) and, even with aggressive treatment, prognosis remains poor. There is no definitive guideline for the treatment of PEL, although CHOP-like regimens (cyclophosphamide, doxorubicin, vincristine, and prednisone) are frequently prescribed and, given the rarity of this disease, therapeutic focus is being redirected to personalized and targeted approaches in the experimental realm. Current clinical trials include the combination of lenalidomide and rituximab into the EPOCH regimen and the treatment of individuals with relapsed/refractory EBV-associated disease with tabelecleucel.

HIV/AIDS Associated Lymphoma: Review

“…Current clinical trials include combining lenalidomide and rituximab in the EPOCH regimen and using tabelecleucel to treat people with relapsed/refractory EBV-associated disease. 91 …”

DSMZCellDive: Diving into high-throughput cell line data

“…Intriguingly, specific B-cell markers get lost in the tumour cells. For example, although CD19 is usually expressed in all B-cells from pre-B-cells until the terminal differentiation to plasma cells 14 , its expression is typically absent in Hodgkin lymphoma (HL), plasma cell leukemia (PCL), primary effusion lymphoma (PEL) and in a fraction of Diffuse large B-cell lymphoma (DLBCL) 15 , 16 . Accordingly, CD19 loss is also seen in cell lines representing HL, PCL, and PEL ( Figure 2C ).…”

HHV-8 associated lymphoma

“…In the past few years, large series from USA and Europe of patients with PEL, HHV-8+ LBCL-NOS or GLPD have provided data that allow a better characterization of these diseases in different populations including HIV infected or not infected patients [18 ▪ ]. Questions regarding possible differences between a number of subgroups can therefore be addressed.…”