CASE PRESENTATION
An 84-years old Jewish woman of North African ancestry presented to the emergency room complaining of effort-related dyspnea, low-grade fever, and a dry cough for several days. her past medical history includes only controlled hypertension. She uses Cinnarizine, Nifedipine, Aspirin, and Atenolol on a regular basis.
Upon arrival, Chest X-ray (Figure 1) showed an enlarged heart silhouette and left lower lobe (LLL) infiltrate. Physical examination revealed diminished breath sounds and pleural dullness during percussion of the left hemithorax.
After several days with no meaningful clinical improvement following treatment with azithromycin and ceftriaxone, CT angiography was performed indicating significant bilateral pleural effusion, moderate pericardial effusion and no lymphadenopathy.
Transthoracic echocardiography (Figure 2) showed a normal function of the left ventricle and large volume of pericardial effusion with a diastolic right-sided collapse. Urgent pericardiocentesis via a subcostal approach was performed and 650 ml of serosanguinous fluid were withdrawn resulting in an immediate relief of symptoms. Pericardial drainage continued for nine days using a pigtailed catheter.
The patient underwent left pleurocentesis with the removal of 500 ml of exudative cloudy fluid. Pleural cell count indicated a total of 42,700 k/ul of white blood cells. Of them, 24% were monocytes, 13% basophils, and 63% were large unstained cells [LUC]. Pleural chemistry analysis showed a significant low glucose level [<11 mg/dL], high protein level [4.36 g/dL], Low pH [6.99], and an extremely high LDH level [44,000, U/L] while Serum LDH was mildly elevated [640 U/L]. The effusion to serum protein ratio was 0.71.
Flow cytometry (NAVIOS machine Beckman Coulter, USA), from the pericardial fluid, analyzed using the side scatter (SSC) gating, indicated 100,000 white blood cells. Of which, 25% were neutrophils (CD13+/CD10+), 25% monocytes (CD64+, CD123-, CD33+) and 12% were from the lymphoid lineage (CD20+, CD3+). An additional CD45- population comprised 38-45% of total cells with the following antigen properties: Kappa+, Lambda-, CD138+, CD38+, CD56+, CD81+, CD229+, CD74+, CD43+, CD19-, CD20-, CD24-, CD54-, CD27-, CD28-, CD200dim, CD117-, CD10-. CD81+ (Figure 3).
Microscopic examination of the fluid revealed large immature atypical lymphocyte. Broad antigens analysis was performed indicating the following: HLA-DR-, CD34-, CD123-, CD33-, CD64-, CD68-, CD4-, cyCd79a-, cyCD3-, MPO-, TdT-.
Flow cytometry of the Pleural fluid showed the same antigenic properties (Figure 4). Immunofluorescent assays (IFA) using the lytic and latent IgG test for the detection of HHV-8 were both positive. Human immunodeficiency virus (HIV) testing using the enzyme-linked immunosorbent assay was negative.
A diagnosis of primary effusion lymphoma was established based on the cytologic findings. suggestive of a lymphoid malignant infiltrate with cells positive for HHV8, along with the peculiar characteristic immunophenotype which was negative for classical B and T cell markers, yet positive for plasma cell markers i.e CD38 and CD138. Additionally, the lack of lymph node or spleen enlargement in whole body CT, and in fact – the lack of any masses at all were all consistent with the diagnosis of PEL.
After consulting with her family members, the patient chose not to start chemotherapy. Her treatment regimen consisted of anti-hypertensive medication (Nifedipine and Bisoprolol), 60mg Prednisone which was slowly tapered over a course of several week, Ibuprofen (1800mg daily for two weeks), 30mg Lansoprazole and 100mg Aspirin.
During the first six months after discharge, she underwent two successful pleural drains. At a two-and-a-half-year follow-up there were no adverse events other than a mild accumulation of pleural fluid that did not require drainage.