Human Herpes Virus 8-unrelated Primary Effusion Lymphoma-like Lymphoma: Report Of A Rare Case And Review Of The Literature
Adiguzel, C., Bozkuright, S. U., Kaygusuz, I., Uzay, A., Tecimer, T., & Bayik, M. (2009). Human herpes virus 8-unrelated primary effusion lymphoma-like lymphoma: report of a rare case and review of the literature. APMIS : acta pathologica, microbiologica, et immunologica Scandinavica, 117(3), 222–229. https://doi.org/10.1111/j.1600-0463.2008.00005.x
View Original Source →Abstract
Primary effusion lymphoma (PEL) is a very rare type of lymphoma usually confined to the body cavities predominantly in immunosupressed patients infected with human herpes virus 8 (HHV‐8). The new term for HHV‐8 independent PEL is HHV8‐unrelated PEL‐like lymphoma. We describe an 89‐year‐old human immunodeficiency virus (HIV)‐negative male patient with HHV8‐unrelated PEL‐like lymphoma in the pleura. No hepatosplenomegaly or lymphadenopathy was detected. Chest radiography and computed tomography revealed right pleural effusion, but no evidence of tumor mass or lymph node enlargement. Cytological analysis of the pleural effusion revealed a high‐grade lymphoma with round nuclei, prominent nucleoli and abundant cytoplasm with immunophenotypes positive for CD45, CD30, CD38, CD7 and CD71. Because of the advanced age, no chemotherapy was given. Effusion resolved spontaneously. One year after the diagnosis, a new pleural effusion developed at the left side. Following thoracentesis and pleurodesis, the patient remained in complete remission for 40 months. To date, 30 cases of HHV8‐unrelated PEL‐like lymphoma/HIV negative have been reported in the literature. The outcome of the HHV8‐unrelated PEL‐like lymphoma patients who were HIV negative seems to be better than HIV‐ and HHV‐8‐positive PEL.
Case Details
Disease Location
Body cavities
Personal Characteristics
89 -year-old male july 2004 had diabetes mellitus, hypertension and coronary arterial disease
Clinical Characteristics
Primary effusion lymphoma (pel) originally admitted with fever, dyspnea, and malaise physical exam found breath sounds on the right hemi thorax were not audible. Chest x-ray and whole-body CT revealed massive right pleural effusion with no evidence of tumor mass or lymph node enlargement. Hemogram revealed wbc count of 6700 cells/ml, hgb of 13 mg/dl and platelet count of 435000/ml. Aspartate aminotransferase level of 32 u/l, alanine aminotransferase 35 u/l, alkaline phophatase 120 u/l, gamma-glutamyltranspepatientidase 60 u/ml, and lactate dehydrogenase 283 u/l. Erythrocyte sedimentation rate was 99 mm/h. Thoracentesis was performed on right effusion. Pleural fluid revealed glucose 157, protein 4.4, and ldh 1519. Cell count showed wbc 4600/ml hgb 0.4 gr/dl, and platelets 8000/ml. Gram stain, ehrlich-ziehl-neelsen and bactec cultures were negative. Cytological evaluation of the pleural effusion found large atypical lymphoid cells with round nuclei, prominent nucleoli and abundant cytoplasm resembling immunoblast and rare reed-sternberg-like cells. Flow cytometric analysis, lumphoma cells were positive for CD45, 30, 38, and CD71, but negative for CD3, 19, 20, 22, 79a, and surface and cytoplasmic immunoglobulin. Immunocytochemistry on cell block of pleural effusion showed atypical lymphoid cells postive for cd30&38. Serologic tests were found to be negative for HIV, HCV and cmv. Hhv-8 and ebv was also negative in pleural fluid by pcr. Patient was discharge 7 days after admin witho chemo. Left pleural effusion has same cytological and flow cytometrics as before. Needle biopsy showed chronic inflammatory cells but neither malignant nor tuberculosis lesions were found.
Remission Characteristics
During follow-up period, symptoms resolved and right pleural effusion resolved. A year later was re-admitted with left side plerual effusion 40 months after initial diagnonsis was without symptoms
Treatment & Mechanisms
Clinical Treatment
Pleural needle biopsy on second effusion pleurodesis after complete drainage of fluid
Non-Clinical Treatment
None reported