Ann Dermatol.  2014 Aug;26(4):496-500. 10.5021/ad.2014.26.4.496.

Intravascular Cytotoxic T-Cell Lymphoma in a Young Immunocompetent Woman

• Jang YH
• Lee SJ
• Choi YH
• Lee WJ
• Kim DW
• Kim J
• Park TI
• Chae YS

• KMID: 2265592
• DOI: http://doi.org/10.5021/ad.2014.26.4.496

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.

Keyword

Human herpesvirus 4; Lymphoma; T-lymphocytes

MeSH Terms

Abdomen
B-Lymphocytes
Biopsy
Breast
Central Nervous System
DNA
Drug Therapy
Female
Gene Rearrangement
Genes, T-Cell Receptor
Granzymes
Herpesvirus 4, Human
Histiocytes
Humans
Immunoglobulin Heavy Chains
In Situ Hybridization
Killer Cells, Natural
Lower Extremity
Lymphocytes
Lymphoma
Lymphoma, T-Cell*
Pathology
Phenotype
RNA
Skin
Stem Cells
T-Lymphocytes
Telangiectasis
Young Adult
DNA
Granzymes
Immunoglobulin Heavy Chains
RNA

Figure

• 

  Fig. 1 A 23-year-old Korean woman with increasing number of erythematous indurated tender patches with telangiectasia over right breast, abdomen, both upper and lower extremities and back for 3 months (inset: close-up view of skin lesion in the right breast).

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  Fig. 2 (A) Large atypical cells fill several deep dermal blood vessels (H&E, ×100). (B) On higher magnification, the tumor cells (black arrows) have irregular hyperchromatic nuclei, prominent nucleoli, and a moderate amount of cytoplasm compared with perivascular lymphocyte populations (H&E, ×400).

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  Fig. 3 The neoplastic cells are positive for CD3 (A, ×400), CD8 (B, ×200), Ki-67 (C, ×400), MPO (D, ×200), and granzyme B (E, ×200). In addition, in situ hybridization study for EBV-encoded RNA was positive (F, ×400).

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