Background: In addition to human T-lymphotropic virus (HTLV-I), the Epstein-Barr virus (EBV) has recently been demonstrated to be associated with cutaneous T-cell lymphoma (CTCL).
Objective: Our purpose was to investigate characteristic clinicopathologic features of the cutaneous lesions of EBV-associated T-cell malignancies.
Methods: Clinical records, laboratory data, and histopathologic sections were reviewed. Freshly frozen tumor tissues were immunophenotyped. Southern blot and in situ hybridization studies were performed to detect the EBV genomes.
Results: Ten of 35 CTCL biopsy specimens collected between 1985 and 1992 were found to be EBV-associated. Clonotypic proliferation of EBV genomes was demonstrated in each case, and the atypical T lymphoid cells contained EBV genomes. The cutaneous eruptions of these patients included multiple violaceous papules or nodules, chronic ulcers, and tumors on the trunk or extremities. Three distinct clinicopathologic subgroups could be recognized. The most consistent was the angiocentric T-cell lymphoma or lymphomatoid granulomatosis (type III CTCL) (four cases), presenting with chronic ulcers or violaceous papules. The second group was the T large-cell lymphoma (type II CTCL), Ki-1 antigen (CD30) (positive or negative) (four cases). Three patients with Ki-1- lymphoma had fulminant disease, whereas the remaining Ki-1+ case had a benign course. The third group was the secondary type CTCL (type V CTCL) (two cases), representing systemic EBV-associated T-cell lymphoma. The prognosis was grave. The common features of these EBV-associated CTCLs are resistance to conventional chemotherapy, poor prognosis, and the terminal manifestation of a hemophagocytic syndrome. No EBV genome could be detected in 12 cases of classic CTCL/mycosis fungoides (type I CTCL), or in three cases of HTLV-I-associated adult T-cell lymphoma (type IV CTCL).
Conclusion: Three distinct clinicopathologic subtypes of EBV-associated CTCL were recognized, including one additional type of virus-associated CTCL.