Kaposi sarcoma herpesvirus/human herpesvirus-8-negative effusion-based lymphoma: report of 3 cases and review of the literature.

[primary effusion lymphoma]

Primary effusion lymphoma (PEL ) is a rare subtype of large B-cell lymphoma that arises in body cavities without detectable tumor masses . PEL is universally associated with Kaposi sarcoma herpesvirus (KSHV )/human herpesvirus-8 (HHV 8 ). Despite overlapping features , KSHV /HHV 8 -negative effusion-based lymphoma is a distinct entity from PEL . To date , 52 cases have been reported . The authors report 3 additional cases received in their laboratory from 2007 to 2012 .Clinical data , cytomorphologic features , and immunophenotypic features of the 3 cases were described and compared with those reported in the literature .The cells in HHV 8 -negative effusion lymphoma commonly revealed large cell , immunoblastic morphology and B-cell immunophenotype . The 3 cases demonstrated cytomorphologic and immunophenotypic variability . Cytomorphologically , 1 case contained large , highly atypical cells with a moderate amount of cytoplasm , round nucleus , coarsely granular chromatin , and a single macronucleolus . The other 2 cases had medium to large atypical cells with high nuclear-to -cytoplasmic ratios , slightly irregular to cleaved nuclei , and multiple conspicuous nucleoli . One case had a null phenotype with aberrant cytokeratin expression . B-cell phenotype was established by clonal immunoglobulin heavy-chain rearrangement using polymerase chain reaction , whereas the other 2 cases demonstrated a B-cell phenotype by flow cytometry and immunohistochemical staining . All 3 cases were negative for both HHV 8 and Epstein-Barr virus .H HV 8 -negative effusion lymphoma exhibits clinical , cytomorphologic , and immunophenotypic variability . Cases with a null-phenotype can be particularly challenging . When effusion lymphoma is suspected , ancillary tests are helpful . Moreover , HHV 8 detection is critical in differentiating PEL and HHV 8 -negative effusion lymphoma , because they have overlapping features yet different prognoses .

Diseases
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Diseases presenting "coarsely granular chromatin" symptom

primary effusion lymphoma

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