CHARACTERISTIC CLINICOPATHOLOGICAL FEATURES OF EPSTEIN-BARR-VIRUS ASSOCIATED PERIPHERAL T-CELL LYMPHOMA

Background. The authors previously reported the existence of a unique subtype of peripheral T-cell lymphoma (PTCL) characterized by a clonot ypical proliferation of Epstein-Barr virus (EBV) in the tumor cells (B lood 1991; 77:799). Detailed clinicopathologic features of this newly recognized entity remain to be clarified. Methods. A retrospective stu dy was done in 23 patients receiving consecutive diagnoses at National Taiwan University Hospital by methods previously described. Results. There were 13 male and 10 female patients, with a median age of 40 yea rs. Seventeen patients had Stage III/IV disease, and 15 patients had f ever as a presenting B symptom. Initial extranodal involvement occurre d in skin (10 patients), lung (4 patients), bone marrow (4 patients), brain (3 patients), and nasal cavity (i patient) and was evidenced by hepatosplenomegaly (6 patients). Sixteen patients had specific histopa thologic features including characteristics similar to angioimmunoblas tic lymphadenopathy with dysproteinemia (3 patients), angioinvasive-ty pe features (6 patients), Hodgkin disease-like features (2 patients), hepatosinusoidal-type features (2 patients), Lennert lymphoma (2 patie nts), and malignant histiocytosis-like features (1 patient). Six (37.5 %) of the 16 patients who received a standard regimen with cyclophosph amide, doxorubicin, vincristine, and prednisone or an equivalent regim en as induction chemotherapy achieved complete remission. The median s urvival time was only 8 months. Six (42.8%) of the 14 patients who hav e died at this report ended up with a terminal hemophagocytosis syndro me. All five relapsed tumors were found to have a strong expression of P-glycoprotein (P-gp). Conclusions. The authors suggest that EBV-asso ciated PTCL should be regarded as a separate entity of non-Hodgkin lym phoma showing characteristic histopathologic features, frequent expres sion of P-gp in relapsed tumor, a terminal hemophagocytosis syndrome, and a generally ominous outcome.