title: Intravascular cytotoxic T-cell lymphoma in a young immunocompetent woman.
abstract: Intravascular lymphoma (IVL) is a rare disorder characterized by the presence of large neoplastic lymphoid cells restricted to the lumens of small vessels with a predilection for the skin and the central nervous system. While the vast majority of cases involving IVL are of B-cell lineage, the disease rarely affects the T-cell, the histiocytes, and the natural killer cells. We report a case of intravascular T-cell lymphoma (IVTL) associated with Epstein-Barr virus (EBV). A 23-year-old healthy woman presented with tender indurated erythematous patches with overlying telangiectasia on her right breast, abdomen, both the upper and the lower extremities and the back for 3 months. The pathology revealed an infiltration of dermal and subcutaneous vessels by large and atypical lymphoid cells with immunohistochemical features of the T-cell lineage with a cytotoxic phenotype (CD3+, CD8+, granzyme B+, TIA-1+, CD4-, CD5-, CD20-, CD56-). Interestingly, the DNA extracted from the skin biopsies demonstrated evidence of a monoclonal immunoglobulin heavy chain gene rearrangement, but no T-cell receptor gene rearrangement was found. In situ hybridization study for EBV-encoded RNA was positive. She was diagnosed with an EBV-associated IVTL. The patient's skin lesions were refractory to the combination of chemotherapy and autologous stem cell transplant, and she expired. The findings in the present case may highlight the unique clinicopathologic aspects of EBV-associated cytotoxic IVTL that occurred in a young, immunocompetent woman.
authors.affiliation: Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Yong Hyun
authors.initials: YH
authors.lastname: Jang
authors.affiliation: Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Seok-Jong
authors.initials: SJ
authors.lastname: Lee
authors.affiliation: Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Yoon Hyuk
authors.initials: YH
authors.lastname: Choi
authors.affiliation: Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Weon Ju
authors.initials: WJ
authors.lastname: Lee
authors.affiliation: Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Do Won
authors.initials: DW
authors.lastname: Kim
authors.affiliation: Department of Pathology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Jeongshik
authors.initials: J
authors.lastname: Kim
authors.affiliation: Department of Pathology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Tae In
authors.initials: TI
authors.lastname: Park
authors.affiliation: Department of Oncology/Hematology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Yee Soo
authors.initials: YS
authors.lastname: Chae
keywords: Human herpesvirus 4; Lymphoma; T-lymphocytes