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Keywords:

  • Epstein-Barr virus;
  • T-cell lymphoma;
  • hemophagocytosis;
  • P-glycoprotein

Abstract

Background. The authors previously reported the existence of a unique subtype of peripheral T-cell lymphoma (PTCL) characterized by a clonotypical proliferation of Epstein-Barr virus (EBV) in the tumor cells (Blood 1991; 77:799). Detailed clinicopathologic features of this newly recognized entity remain to be clarified.

Methods. A retrospective study 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 years. Seventeen patients had Stage III/IV disease, and 15 patients had fever as a presenting B symptom. Initial extranodal involvement occurred in skin (10 patients), lung (4 patients), bone marrow (4 patients), brain (3 patients), and nasal cavity (1 patient) and was evidenced by hepatosplenomegaly (6 patients). Sixteen patients had specific histopathologic features including characteristics similar to angioimmunoblastic lymphadenopathy with dysproteinemia (3 patients), angioinvasive-type features (6 patients), Hodgkin disease–like features (2 patients), hepatosinusoidal-type features (2 patients), Lennert lymphoma (2 patients), and malignant histiocytosis-like features (1 patient). Six (37.5%) of the 16 patients who received a standard regimen with cyclophosphamide, doxorubicin, vincristine, and prednisone or an equivalent regimen as induction chemotherapy achieved complete remission. The median survival time was only 8 months. Six (42.8%) of the 14 patients who have died at this report ended up with a terminal hemophagocytosis syndrome. All five relapsed tumors were found to have a strong expression of P-glycoprotein (P-gp).

Conclusions. The authors suggest that EBV-associated PTCL should be regarded as a separate entity of non-Hodgkin lymphoma showing characteristic histopathologic features, frequent expression of P-gp in relapsed tumor, a terminal hemophagocytosis syndrome, and a generally ominous outcome.