INTRODUCTION:Primary effusion lymphoma (PEL) grows as a lymphomatous effusion with unique pathogical characteristics but without an identifiable primary solid tumor.
CASE PRESENTATION:A 38 year-old male with HIV/AIDS (CD4 count of 3 lymphocytes/mL, on no antiretroviral medication), and recent Pneumocystis jirovecii pneumonia (PCP), Hepatitis C virus, and polysubstance abuse was admitted with one month of progressive cough and dyspnea and weight loss of fifteen pounds. He denied fever or chest pain. On physical examination, the patient appeared cachectic, anxious, and alert. The temperature 102°F orally, blood pressure 95/51, pulse 112/minute, respirations 22/minute, and O2 saturation of 92% on 3L nasal cannula. There was pallor, bilateral wheezing and rhonchi, splenomegaly, and shallow perianal ulcerations. There was no icterus, thrush, lymphadenopathy, jugular venous distension, abnormal heart sounds, hepatomegaly, abdominal tenderness, edema, clubbing, or focal neurological deficit. Initial labs on admission revealed pancytopenia (platelet 21, hemoglobin 9, WBC 0.6), hyponatremia (127) and deranged LFTs (albumin 2.2, SGOT 99, SGPT 90, alkaline phosphatase 171). LDH was 236. Initial ABG was PH 7.46, pCO2 29, pO2 64 on 3L nasal cannula. Chest X-Ray revealed right pleural effusion (figure 1). Intravenous trimethoprim/sulfamethoxazole (TMP/SMX), prednisone, ceftriaxone, azithromycin and valacyclovir were begun empirically. Thrombocytopenia worsened and clindamycin/primaquine were substituted for TMP/SMX. PPD and AFB sputum smears were negative. Patient refused bronchoscopy and thoracentesis. Two days later, he developed progressive hypoxia and tachycardia with worsening effusion on X-ray. Transthoracic echocardiography revealed cardiac tamponade physiology and nodular pericardial thickening.Computer tomography of the chest revealed evidence of tampanode (Fig 2). A pigtail catheter was placed in to the right pleural space, fluid analysis revealed lymphocytic exudate with an LDH of 4706 IU. Follow up echocardiography showed resolution of the tamponade physiology. Fevers persisted with repeated negative cultures inspite of broad spectrum antibiotics. Cytologic examination of the pleural fluid revealed atypical lymphoid cells and abundant apoptotic cells. Flow cytometry showed atypical mature T-cells with enlarged nuclei and open chromatin, expressing CD-2, 3, 5, 7, and 8 but not CD-4, 56, 34 nor granulocytic markers. On hospital day 13, he became hemodynamically unstable and repeat echocardiogram revealed tamponade physiology. Pericardial window was done and a large-bore chest tube into the right chest was inserted. Despite aggressive care, sepsis with multi-organ failure developed and the patient expired on hospital day 24.
DISCUSSIONS:We found no published cases of T-cell PEL in HIV/AIDS. The flow cytometric data and cardiovascular complications of this case are extremely unusual. PEL (or body cavity lymphoma) is a lymphomatous effusion of the pleural, pericardial or peritoneal cavity without identifiable tumor mass. Prognosis is poor, with median survival of 4 months after diagnosis. In 1996 Nador et. al. detected Kaposis sarcoma-associated herpes virus (KSHV/HHV8) in PEL. Unique features of PEL include: 1. A liquid rather than a solid proliferation of malignant lymphocytes confined to growth within a major body cavity; 2. A large-cell, immunoblastic, plasmablastic, or anaplastic cytomorphology; 3. Indeterminate immunophenotype by nonmolecular methods; 4. A tendency to develop in male AIDS patients who have sex with men, likely because of human herpesvirus-8. PEL accounts for approximately 3% of AIDSrelated lymphomas and 1% of all non-AIDS related large cell lymphomas. Flow cytometry of serous effusions can be a useful adjunct to conventional cytopathology. PCR amplification can be utilized to detect KSHV in PEL. KSHV testing of this patient’s malignancy is in process.
CONCLUSION:HIV-associated T-cell PEL has not yet been reported in the published literature. This case underlines the utility of flow cytometry in the workup of lymphomatous effusions and the serious hemodynamic effects of massive pleural effusions on cardiac function.
DISCLOSURE:Anuja Garg, No Financial Disclosure Information; No Product/Research Disclosure Information