Primary Effusion Lymphoma (PEL) in HIV-Negative Patients - a Distinct Clinical Entity

Abstract

Primary effusion lymphoma (PEL) is a recently described rare type of non-Hodgkin's lymphoma occurring almost exclusively in HIV infected people. Human herpesvirus 8 (HHV-8), has been linked with PEL, and a causative relationship has been suggested. In the vast majority of PEL cases Epstein-Barr virus (EBV) has been found in the tumour cells. We describe here an elderly human immune deficiency (HIV) seronegative man with intractable chest pain and pleural effusion. The diagnosis of malignant lymphoma was suggested cytologically and confirmed histologically following pleural biopsy. No lymphadenopathy or organ involvement with lymphoma was found. Systemic chemotherapy with a modified CHOP regimen with G-CSF support gradually led to the resolution of the chest pain and ultimately resulted in a complete clinical remission (CCR). The presence of HHV-8 was demonstrated by PCR using paraffin-embedded tissue samples from the involved pleura, whereas EBV-associated genetic material was absent. The patient remained in CCR for 18 months and died of an unrelated cause (cerebrovascular event). Only 11 other cases with clinical and virological features similar to those of our patient have been reported in the literature. Analysis of these rare cases suggests HIV-negative EBV-negative PEL to be a distinct clinical entity with epidemiological features resembling classical KS and supports an EBV-independent role for HHV-8 in the pathogenesis of PEL.

Combined chemotherapy including high‐dose methotrexate in KSHV/HHV8‐associated primary effusion lymphoma

“…PEL has been rarely observed in HIV-negative patients, and the relatively old age of these patients and/or the presence of visceral failures hampered the use of cytotoxic drugs [11,12]. CHOP has been the most frequently used chemotherapy regimen [2,4,6,7,[13][14][15]. Low-dose m-BACOD [4,6] and COP [16] have been occasionally administered.…”

“…The main causes of death were PEL progression, visceral KS, and severe sepsis. Late deaths related to long-term toxicities of anthracyclines have also been reported [15,17]. The achievement of a long-term CR after CHOP has been reported in only 2 patients [6,14].…”

The proteasome inhibitor bortezomib (PS-341) inhibits growth and induces apoptosis in primary effusion lymphoma cells

“…2,3 PEL mainly occurs in AIDS patients and less frequently in other groups, including organ transplantation recipients and elderly patients. [4][5][6][7] Due to the existence of comorbid conditions and poor performance status, patients with PEL are in general poor candidates for aggressive chemotherapy and median life expectancy of patients treated with standard chemotherapy is approximately 2 months. 8,9 The lack of substantial progress in the treatment of PEL over the past decade indicates the need for novel therapeutic approaches.…”

Curcumin suppresses growth and induces apoptosis in primary effusion lymphoma

“…Primary effusion lymphoma (PEL) is a subtype of nonHodgkin's B-cell lymphoma, that mainly presents in patients with advanced AIDS, but is sometimes also found in human immunodeficiency virus (HIV)-negative individuals (Nador et al, 1996;Klepfish et al, 2001). PEL cells grow as a lymphomatous effusion in body cavities and are infected with Kaposi sarcoma-associated herpesvirus (KSVH/HHV8).…”