皮肤血管内NK/T细胞淋巴瘤一例

患者女，18岁。双小腿皮肤红斑、结节伴疼痛2年余。皮肤科检查：左小腿内后侧弥漫性浸润性暗红斑，红斑范围内可触及深在大小不等皮下结节，质稍硬，伴显著触痛。皮损组织病理检查：表皮未见明显异常，真皮及皮下血管周围淋巴样细胞浸润，多处血管壁坏死及血管闭塞，部分血管内可见成团的淋巴细胞，其中可见较多核大浓染异形细胞及病理性核分裂。免疫组化染色：血管内淋巴细胞CD3（+++）、CD3ε（+++）、CD2（+）、CD56（+++）、粒酶B （+++）、穿孔素（+++）、CD30（+），Ki67（+++，100%），CD20、CD5、CD7、CD4、CD8、TdT、间变性淋巴瘤激酶、上皮膜抗原、泛细胞角蛋白均为阴性，CD34示血管（+），原位杂交显示血管内淋巴细胞EB病毒编码RNA1/2（+）。诊断：皮肤血管内NK/T细胞淋巴瘤。

Cutaneous intravascular NK/T cell lymphoma: a case report

An 18-year-old female presented with painful erythema and nodules on both legs for more than 2 years. Dermatological examination showed irregularly sized, mildly indurated, tender, deep subcutaneous nodules arising in diffused infiltrated dark erythematous patches in the inner and posterior region of the left leg. Histopatho-logy showed no significant changes in the epidermis. There were perivascular lymphoid cell infiltrates in the dermis and subcutis. Multiple sites of necrosis of blood vessel walls with vascular occlusions were noted. The lumens of some blood vessels were filled with lymphocytes, among which were many atypical cells with hyperchromatic nuclei and pathologic mitotic figures. Immunohistochemistry showed that lymphocytes in the cavities of blood vessels were positive for CD3（+++）, CD3ε（+++）, CD2（+）, CD56（+++）, granzyme B（+++）, perforin（+++）, CD30（+），Ki67（+++, 100%）, but negative for CD20, CD5, CD7, CD4, CD8, TdT, anaplastic lymphoma kinase, early membrane antigen （EMA） or pan cytokeratin （pCK）. The endothelial cells lining the blood vessels stained positively for CD34. The intravascular lymphocytes were also positive for EBER1/2 by in situ hybridization. A diagnosis of cutaneous intravascular NK/T cell lymphoma was made.