A 44 year-old man with history of anemia and HIV on antiretroviral therapy (ART) with CD4 359 presented with a 3-month history of progressive bilateral lower extremity swelling, increasing abdominal girth, and scrotal edema. He reported a 20-pound unintentional weight loss over the last year, associated with progressive fatigue and drenching night sweats for 3 months. He denied fever, headaches, abdominal pain, or changes in bowel habits. Physical exam revealed a pale cachectic male with cervical and axillary lymphadenopathy, tachycardia, distended tense abdomen with palpable spleen and fluid wave, 2+ pitting lower extremity edema, and scrotal edema. Initial laboratory evaluation was significant for normocytic anemia 6.5g/dL, thrombocytopenia 126k/uL, and hypoalbuminemia 1.6g/dL. Further testing showed positive EBV and HHV-8 antibodies. CT-scan showed subcentimeter axillary, mediastinal and retroperitoneal lymphadenopathy, moderate right-sided pleural effusion, small bowel mass, splenomegaly, and ascites. Multiple lymph node biopsies were non-diagnostic. Thoracentesis was performed for worsening dyspnea showing atypical cells positive for HHV-8, BOB-1, CD45, CD30 and negative for CD20 and PAX5, consistent with primary effusion lymphoma (PEL). Ascitic fluid cytology was also consistent with PEL. Bone marrow aspirate showed hypercellular marrow with trilineage hematopoiesis and reactive T-lymphocytes. The patient was diagnosed with HIV and HHV-8-associated PEL. Given his poor performance status, ECOG 4, treatment with bortezomib and valganciclovir was started. Despite initiation of chemotherapy, the patient continued to deteriorate and comfort measures were eventually pursued.
Discussion:
HIV infection results in impaired cellular immunity leading to an increased incidence of a wide range of malignancies. PEL is a rare and aggressive subtype of HIV-associated non-Hodgkin lymphoma (NHL) that only accounts for 1-4% of NHL cases. PEL manifests as malignant effusions without extracavitary involvement. As such, symptoms are related to fluid accumulation, such as dyspnea from pleural or pericardial effusions, and abdominal distension from ascites. PEL is associated with EBV+, however it is distinguished from all of other lymphomas by its HHV-8 positivity. Due to its rarity, there is no therapeutic standard of care for PEL. Current options are based on case reports or in vitro data for which currently PEL remains a diagnosis with extremely poor prognosis. Aggressive chemotherapy with EPOCH or CHOP in combination with ART has shown the most success with a median overall survival of 6-months. However, as in our case, therapy selection is limited by patient’s performance status and side effect tolerance.
Conclusions:
As the lifespan of HIV+ patients has increased in the era of ART, the mortality associated with malignancies has also increased. Hence, hospitalists should consider HIV-associated malignancies, such as PEL, in the differential of HIV+ patients with nonspecific or infectious-like complaints.