Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis.

Abstract

Primary effusion lymphoma (PEL) is a human herpes virus-8 (HHV-8)-associated and very rare type of lymphoma usually confined to the body cavities and commonly observed in human immunodeficiency virus (HIV)-infected patients. A comparison was made between the cytologic and immunocytochemical features of 4 cases of PEL encountered in the authors' department with those reported to date in the literature. A comprehensive comparison of the cytologic and immunocytochemical features of the 4 cases with those reported in the literature was conducted. Cytologically, the most consistent features of the 4 cases and those in the literature included large cell size, moderate to abundant cytoplasm, a single nucleus in most cells with occasional bi- or multinucleated giant cells, single to multiple prominent nucleoli, and coarse chromatin. Immunocytochemically, only 2 (50%) of the current cases were of the null-phenotype compared with 93% of cases in the literature; the other 2 cases had a T-cell phenotype. Activation markers were expressed in 50% and 78% of the current cases and the literature cases, respectively. Positivity for HHV-8 was proven in the 4 cases by immunocytochemistry. Cytomorphologically, PEL exhibits features bridging large cell immunoblastic and anaplastic large cell lymphoma. Although it is usually of null-phenotype, it may occasionally express B-cell or T-cell markers, rendering its distinction difficult from other lymphomatous effusions on a cytologic and immunocytochemical basis alone. Therefore, HHV-8 detection is an essential confirmatory ancillary test in suspected cases of PEL.