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目的报道1例特发性免疫球蛋白缺乏症继发外周T细胞淋巴瘤。方法通过病史、临床及实验室检查,病理及免疫组化以排除其他肿瘤性疾病,并观察临床用药治疗效果。结果25岁男性患者的皮损呈丘疹、斑块及结节.发生于左臀部、上唇、鼻翼等部位。主要表现为反复发生的严重感染.IgG明显低于正常水平(<0.069 g/L)。皮损组织病理检查见真皮内大量大小不等的淋巴细胞。呈弥漫性分布.核异形,有丝分裂明显。皮损免疫组化检查.CD7、CD2、CD4、LAT均阳性,Ki-1、CD20、OCT2、CD56、CD68、CD79a均阴性。结论本患者的诊断主要依据相关的实验室检查.临床表现是本病诊断的重要线索.有助于疾病的早期诊断。
Objective To report a case of idiopathic immunoglobulin deficiency secondary peripheral T cell lymphoma. Methods By history, clinical and laboratory tests, pathology and immunohistochemistry to exclude other neoplastic diseases, and observe the effect of clinical drug treatment. Results The skin lesions in 25-year-old men were papules, plaques and nodules, which occurred in the left hip, upper lip, nose and other parts. The main manifestations of recurrent severe infection.IgG was significantly lower than normal (<0.069 g / L). Skin lesions histopathology see a large number of different sizes of lymphocytes in the dermis. A diffuse distribution of nuclear heterotrophic, mitotic obvious. Immunohistochemical examination showed that CD7, CD2, CD4 and LAT were all positive, Ki-1, CD20, OCT2, CD56, CD68 and CD79a were all negative. Conclusion The diagnosis of this patient is mainly based on the relevant laboratory tests, and clinical manifestations are important clues to the diagnosis of this disease and contribute to the early diagnosis of the disease.