An 81-year-old male with coronary artery disease, valvular heart disease status post mitral valve replacement and tricuspid annuloplasty ring implant, coronary artery bypass graft, persistent atrial fibrillation, hypertension, and congestive heart failure with preserved ejection fraction (EF 60%) presented to his cardiologist's office complaining of increased fatigue, cough, orthopnea, and exertional dyspnea for 3-4 weeks. He denied any significant weight loss, drenching sweats, increased lymph node size, fevers, or chills. Eight months prior to current presentation during hospitalization for a heart failure exacerbation, the patient was noted to have small bilateral pleural effusions (left greater than right) and underwent left-sided thoracentesis at that time, which revealed no evidence of infection or malignancy. Only one month following that time, the patient underwent mitral valve replacement surgery and during the procedure, the patient underwent bilateral drainage for re-accumulating pleural effusions. Only one month prior to current presentation, the patient was again noted to have another right-sided pleural effusion accredited to heart failure, and the decision was made to up-titrate the patient's diuretic regimen instead of undergoing thoracentesis.
Upon current presentation, chest x-ray reconfirmed a large right-sided pleural effusion, and the patient subsequently underwent repeat diagnostic and therapeutic thoracentesis with serous fluid removed. Cytology was positive for large lymphoma cells (Figure 1A and B). By immunostaining, lymphoma cells were positive for CD20, PAX-5, BCL-2, BCL-6, and MUM-1, and were negative for CD138, CD10, and MYC. The Ki-67 proliferation index was high at >90% (Figure 1C-H). Fluorescence in situ hybridization (FISH) testing on lymphoma cells revealed that 55% of nuclei had three intact copies of MYC located at 8q24, indicative of trisomy 8 or an 8q duplication. Pathologic findings were consistent with NGCB-DLBCL. An extensive infectious workup, including Epstein-Barr virus (EBV) in situ hybridization testing, HHV-8 immunostaining, hepatitis C virus RNA quantitative serum testing, hepatitis B core antibody testing, HIV antibody screening, and QuantiFERON Tb-gold analysis were all negative. Computed tomography (CT) scan of the chest, abdomen, and pelvis showed no evidence of pleural-based abnormalities or lymphadenopathy. Full-body positron emission tomography (PET) scan further demonstrated no evidence for any fluorodeoxyglucose (FDG) avid process (Figure 2). Bone marrow aspirate and biopsy was negative for lymphoma. The diagnosis of primary effusion-based lymphoma (PEBL) was made.
Due to the patient's age and with concern towards his ability to tolerate standard anthracycline-based chemoimmunotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), the patient was initially treated with one dose of rituximab (375mg/m2) as single agent therapy followed by rituximab and cyclophosphamide (750mg/m2) for two cycles, which were well tolerated. He was then transitioned to R-CHOP with a 30% reduction in doxorubicin dosing due to concerns for cardiac toxicity. Before the second cycle of R-CHOP, he was noted to have a persistent moderate right-sided pleural effusion, and he underwent thoracentesis with negative cytology for lymphoma cells. The patient subsequently completed an additional 5 cycles of R-CHOP (for a total of 6 cycles). CT chest, abdomen, and pelvis following treatment completion revealed no suspicious lymphadenopathy with a small stable right-sided pleural effusion. Thereafter, the patient was monitored with surveillance imaging every 4-6 months and did not require any further thoracenteses due to resolution of his previous pleural effusion. Currently, he remains in complete remission 51/2 years after the time of his original diagnosis.