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目的报告一例罕见的以环形红斑为初发表现的成人T淋巴母细胞性淋巴瘤。方法对其临床、组织病理、免疫组化、分子生物学和各种特殊的实验室检查进行研究。结果颈部、腹股沟肿大数10个淋巴结,全身百余个环形红斑。胸部CT示:前上纵隔占位性淋巴瘤表现。胸水中找到异形细胞。骨髓片示:淋巴瘤累及骨髓。外周血中未发现瘤细胞。淋巴结结构破坏,瘤细胞弥漫分布,呈“满天星”图像,部分瘤细胞细胞核呈曲核型;免疫组化示:LCA、CD43、UCHL鄄1、TdT均阳性。皮肤损害示真皮浅层带状淋巴样细胞浸润,部分向表皮性,可见Pautrier微脓肿,少数淋巴样细胞细胞核呈曲核型,与淋巴结的瘤细胞相似;免疫组化示:LCA、CD43、UCHL鄄1均阳性,TdT阴性。原位杂交示EBER1/2在淋巴结组织中呈阳性;皮肤组织中呈阴性。结论此例为首例报告罕见的以环形红斑为初发表现的T淋巴母细胞性淋巴瘤。
Objective To report a rare case of adult T-lymphoblastic lymphoma with ring-shaped erythema as the first manifestation. Methods The clinical, histopathological, immunohistochemical, molecular biology and various special laboratory tests were studied. Results of the neck, groin enlargement number 10 lymph nodes, the body more than 100 ring erythema. Chest CT showed: anterior mediastinal lymphoma performance. Pleural effusion found in abnormal cells. Bone marrow shows: lymphoma involving the bone marrow. No tumor cells were found in peripheral blood. Lymph node structure destruction, diffuse distribution of tumor cells, was “gypsophila” image, part of the tumor cell nuclei were karyotype; immunohistochemistry showed: LCA, CD43, UCHL Juan 1, TdT were positive. Skin lesions showed the superficial zonal lymphoid cells infiltrating into the dermis. Some of them showed epidermis, showing Pautrier micro abscess. The nuclei of a few lymphoid cells showed karyotype, which was similar to that of lymph node. Immunohistochemistry showed that LCA, CD43, UCHL Juan 1 were positive, TdT negative. In situ hybridization showed EBER1 / 2 was positive in lymph node tissue; skin tissue was negative. Conclusions This case is the first case of T lymphoblastic lymphoma with a rare case of erythema rosette.