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.

journal_name

Leuk Lymphoma

journal_title

Leukemia & lymphoma

authors

Klepfish A,Sarid R,Shtalrid M,Shvidel L,Berrebi A,Schattner A

doi

10.3109/10428190109058002

keywords:

subject

Has Abstract

pub_date

2001-04-01 00:00:00

pages

439-43

issue

3-4

eissn

1042-8194

issn

1029-2403

pii

I308J001183

journal_volume

41

pub_type

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