A 39-year-old Hispanic male presented with one week of increasing shortness of breath and enlarged lymph nodes in the neck. He had purple lesions on his shoulders which appeared a few months before the onset of the shortness of breath. On physical examination, his vitals were normal, he had palpable cervical, supraclavicular, axillary, inguinal lymph nodes. His skin showed multiple hard purple nodules. His rapid HIV test was positive with a low CD4 cell count of 129/uL.

CT chest showed patient had diffuse lymphadenopathy with moderate bilateral pleural effusion.The skin nodules were biopsied and showed Kaposi sarcoma (depicted below)

  The final diagnosis was primary effusion lymphoma (PEL) and Kaposi sarcoma. Patient was treated on an AIDS Malignancy Consortium (AMC) clinical trial with da-EPOCHR plus vorinostat, AMC 075. 

CBC Data:

• WBC.......5.2 K/uL
• Hgb.......10.7 g/dL
• MCV.......86 fL
• Platelets.......109 K/uL

Learning points:

1. The case depicts a typical primary effusion lymphoma occuring in the setting of HIV. In this setting, these are near-universally positive for HHV8/KSHV/LANA-1 as depicted here.
2. These lymphomas exhibit plasmablastic cytomorphology and may be aberrrantly CD3+. They are positive for EBV and HHV8 (similar to germinotropic LPD cases) in contrast to KSHV-related Multicentric Castleman disease (KSHV-MCD) which is usually positive only for HHV8 without EBV coinfection.