Abstract:

A 51-year-old man presented with a 4-month history of upper abdominal distending pain and 1-month history of cutaneous nodules and plaques on the neck, trunk and bilateral thighs. The patient underwent many gastrointestinal tract examinations in several local hospitals, and symptomatic treatment did not work. The biopsy of nodules on the abdomen revealed medium- to large-sized atypical lymphoid cells within numerous small vessels in lower dermis and subcutaneous fat tissue. Additionally, the atypical cells were present exclusively within vascular lumina. Immunohistochemical labeling showed the reactivity of neoplastic cells to CD2, CD99, CD3ε, CD43, CD56, Epstein-Barr virus-encoded small nuclear RNAs （EBER）, and cytotoxic proteins such as T-cell intracellular antigen-1 （TIA-1） and perforin, but not to CD4, CD8, CD20, CD79a, CD30, cytokeratin （CK）, S100, or CD68. The endothelial cells lining the involved vessels exhibited the reactivity to CD31 and CD34. Based on the above findings, the patient was diagnosed with intravascular NK/T-cell lymphoma firstly manifesting as gastrointestinal tract symptom and complicated by skin lesions. Following combined chemotherapy with cyclophosphamide, daunorubicin, vincristine, prednisone and etoposide, the patient experienced a quick and satisfactory recovery and the follow-up still continued.

Key words: NK/T-cell