皮肌炎并发结外NK/T细胞淋巴瘤1例

A Case of Extranodal NK/T Cell Lymphoma with Dermatomyositis

患者女,56岁。4年前小腿出现红斑并发皮下结节,1年前出现面部肿胀、四肢肌肉酸痛,伴LDH(659IU/L)、GOT(165IU/L)、CPK(551IU/L)升高。皮损组织病理示:基底细胞液化变性,真皮和皮下脂肪内较多淋巴样细胞浸润,有异形,部分围绕血管,管壁有破坏。免疫组化示:CD3(-),CD2(+),CD4(-/+),CD8(-/+),CD30(-/+),CD56(+),TIA(+),GranzymeB(+),Perforin(+)。肌肉活检见少量肌纤维肿胀,肌横纹不清,肌核增多,肌束间少量淋巴细胞浸润。肺HRCT示肺间质性改变。诊断:结外NK/T细胞淋巴瘤;皮肌炎。皮肌炎并发皮肤T细胞淋巴瘤报道少见。皮肌炎的皮下结节有并发结外NK/T细胞淋巴瘤的可能,应进行组织病理检查予以排除。

A 56-year-old female presented with repeated erythema, subcutaneous nodules on the legs for four years. She had been suffering from face swelling, muscle soreness for one year, accompany with elevation of LDH, GOT, CPK. The histopathology of the cutaneous lesion represented basal ceils liquefied degeneration and in- filtration of heterotypic lymphoidocytes within the dermal and subcutaneous adipose layer. Blood vessels were destroyed and surrounded with heterotypic lymphocytes. Immunohistochemical staining showed CD3 - , CD2 ＋, CIM -/＋, CD8 -/＋ , CD30 -/＋ , CD56 ＋ , TIA ＋ , GranzymeB ＋ and Perforin ＋. Histopathologic findings of the muscle biopsy represented muscle fiber swelling, muscle stripes indistinct, myonuclear in- crease and perifascicular infiltrated with small amount of lymphocytes. High-resolution CT （HRCT） scan- ning displayed interstitial lung disease. The diagnosis of extranodal NK/T-cell lymphoma with dermatomyosi- tis was made. The concomitant of dermatomyositis and cutaneous T cell lymphoma was rarely reported. The lesion of nodules of patient with dermatomyositis highlights the need of skin biopsy to exclude the possibility of extranodal NK/T-cell lymphoma.