伴噬血细胞综合征的皮肤NK/T细胞淋巴瘤鼻型一例

患者男，48岁，反复面颈、躯干、四肢丘疱疹10年，加重10 d，发热4 d。皮损组织病理检查示真皮内大量小到中等异形淋巴样细胞浸润，以血管及附属器周围明显，伴血管结构的破坏，并可累及皮下脂肪小叶间隔。免疫组化标记示真皮及皮下组织浸润的异形细胞CD45RO（+++）、CD3（-）、CD4（-）、CD8（+）、CD56（+）、TIA-1（++）、粒酶B（+）、EBER（++）、CD3ε胞质（++）、CD20（-）、CD79a（-）、CD30（-）。实验室检查示外周血红细胞、白细胞、血小板进行性下降，转氨酶、胆红素持续性升高，高甘油三酯血症及纤维蛋白原降低，腹部B超示肝脾肿大。诊断：皮肤NK/T细胞淋巴瘤鼻型伴噬血细胞综合征。

A case of extranodal NK/T-cell lymphoma, nasal type complicated by hemophagocytic syndrome

A 48-year-old man presented with a 4-day history of fever and 10-year history of papulovesicles on the face, neck, trunk and limbs which had been aggravated 10 days prior to the presentation.Skin biopsy showed a dermal infiltration of numerous small- to medium-sized atypical lymphocytes, which was mainly located around blood vessels or appendages, with the involvement of subcutaneous fat tissue and destruction of blood vessels. The infiltrating atypical cells stained positive for CD45RO, CD8, CD56, T-cell intracellular antigen-1, granzyme B, Epstein-Barr virus-encoded small nuclear RNAs (EBER), but negative for CD20, CD79a, CD3, CD4 or CD30. Cytoplasmic CD3ε was also observed in these cells. Laboratory examinations on admission revealed a progressive decrease in peripheral erythrocytes, white cells and platelets, persistent increase in serum aminotransferase and bilirubin, and decline in serum fibrinogen and hypertriglyceridemia. The B-mode ultrasound of the abdomen showed hepatosplenomegaly. Based on the above findings,the diagnosis was made as extranodal nasal type NK/T-cell lymphoma of skin complicated by hemophagocytic syndrome.