J Pathol Transl Med.  2023 Nov;57(6):332-336. 10.4132/jptm.2023.10.30.

Intravascular NK/T-cell lymphoma: a case report and literature review

• Na JM
• Jung W
• Kim M
• Cheon YH
• Lee JS
• Song DH
• Yang JW

• KMID: 2547931
• DOI: http://doi.org/10.4132/jptm.2023.10.30

Abstract

Intravascular lymphoma is characterized by an exclusively intravascular distribution of tumor cells. Intravascular natural killer/T-cell lymphoma (IVNKTL) is extremely rare, highly aggressive, commonly Epstein-Barr virus (EBV)–positive, and predominantly affects the skin and central nervous system. Here we report a case of IVNKTL diagnosed in a 67-year-old female, presenting with persistent intermittent fever and skin rashes throughout the body. Incisional biopsy of an erythematous lesion on the chest exhibited aggregation of medium to large-sized atypical lymphoid cells confined to the lumen of small vessels that were positive for CD3, granzyme B, and CD56 on immunohistochemistry and EBV-encoded RNA in situ hybridization. EBV DNA was also detected in serum after diagnosis. With a review of 26 cases of IVNKTL to date, we suggest that active biopsy based on EBV DNA detection may facilitate early diagnosis of IVNKTL.

Keyword

Malignant lymphoma; Extranodal NK-T-cell lymphoma; Epstein-Barr virus

Figure

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  Fig. 1. Microscopic findings of cutaneous intravascular natural killer/T-cell lymphoma. Medium-sized atypical lymphoid cells can be seen in subcutaneous vessels (A). CD31 highlights vasculature (B), and atypical cells are positive for CD3 (C), granzyme B (D), and Epstein-Barr virus– encoded RNA in situ hybridization (E). CD56 shows weak positivity in atypical cells (F). CD20 (G), CD4 (H), CD8 (I), and CD30 (J) are negative. Ki-67 index is more than 90% (K).

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