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Discussion
Case Presentation
References
• A 91-year-old man with atrial fibrillation on
coumadin presented with worsening dyspnea.
• Chest X-ray demonstrated large, bilateral
pleural effusions. Echocardiogram showed a
large free flowing pericardial effusion with signs
of early tamponade. Laboratory analysis
showed an INR of 6 and a normal leucocyte
count.
• Pericardiocentesis removed 265 cc of
hemorrhagic fluid. The cytology was negative for
malignancy. Thoracentesis was performed
draining 600 cc of hemorrhagic fluid from the
right, and 1100 cc from the left. The pleural fluid
was exudative with a predominance of large
lymphocytes.
• Cytology and flow cytometry established the
diagnosis of diffuse large B cell lymphoma.
• HHV–8 staining and HIV testing were
negative. The lymphoma cells demonstrated
moderate CD20 expression. A torso CT scan
showed no lymphadenopathy.
• He is being treated with rituximab/bortezomib
and his effusions have not returned since
starting therapy 8 months ago.
1. Siddiqi T et al A case of HIV-negative primary
effusion lymphoma treated with bortezomib,
pegylated liposomal doxorubicin, and rituximab.
Clin Lymphoma Myeloma 2008; 8:300-4.
3. Takahashi et al HIV-negative, HHV-8-
unrelated primary effusion lymphoma-like
lymphoma: report of two cases Am J Hematol
2010 Jan; 85(1):85-7
3. An J et al. Antitumor effects of bortezomib
(PS-341) on primary effusion lymphomas.
Leukemia 2004; 18:1699-704
Image 1 : Chest X-ray AP view on admission showing
bilateral pleural effusions (left), post thoracentesis (right)
Primary effusion lymphoma (PEL) is a rare type
of non-Hodgkin's lymphoma confined to body
cavities. It is often associated with human
immunodeficiency virus (HIV) and herpes virus
8 (HHV-8) infection. We report a rare case of
PEL in an elderly HIV-negative man.
• PEL is a rare and aggressive lymphoma seen
primarily in HIV patients. There are
approximately 30 cases of HIV-negative PEL
reported, mostly associated with advanced age.
• PEL carries a poor prognosis with median
survival of 2-3 months and up to 6 months with
aggressive chemotherapy.
• There is little published data regarding the
treatment of PEL. HIV-negative, HHV-8-
unrelated PEL can express B-cell markers and
has previously been reported to respond to
rituximab.
• A case of successful treatment of HIV-negative
PEL with bortezomib, pegylated doxorubicin and
rituximab has been published.
• Prior reports have suggested that HIV-
negative, HHV8-unrelated PEL has a more
favorable outcome than HIV-associated disease.
• We are encouraged by our patient’s initial
response to treatment
Chest X-rays
Image 2 : Chest CT (left) 3 months post treatment
(right) 8 months post treatment showing resolution of
effusions
A Case Of Primary Effusion Lymphoma Not Associated With Human
Immunodeficiency Virus (HIV) Or Human Herpes Virus 8 (HHV-8)
Jonelle A. Raphael, M.D., Rajitha Sunkara, M.D., Rania Esteitie, M.D.,
Anuj Mittal, M.D., Dorcas Chi, M.D., Peter LaCamera, M.D.
Steward St. Elizabeth's Medical Center, Boston, MA,
Tufts University School of Medicine, Boston, MA
Introduction
Follow up Chest CTs
Fluid analysis
Fluid Type Pericardial Right Pleural Left Pleural
WBC / cmm 2837 11612 6333
RBC / cmm 1118000 468000 67000
PMN% 20 3 2
Lymphocytes % 73 88 89
Monocytes% 7 9 9
Total protein g/dl 3.6 3.5 3.3
Albumin g/dl 1.5 1.6 1.6
LDH U/L 1309 2265 1524
Glucose mg/dl 52 18 82