Cheng G, Zhang X, Yang F, Cheng J Y, Liu Y Y
Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China.
Department of Department of Pathology, Peking University People's Hospital, Beijing 100044, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2020 Dec 18;52(6):1150-1152. doi: 10.19723/j.issn.1671-167X.2020.06.028.
Angioimmunoblastic T-cell lymphoma is a rare T-cell lymphoma. The clinical manifestations are not specific. In addition to the common clinical manifestations of lymphomas such as fever, weight loss, night sweats and lymphadenopathy, it may also have skin rashes, arthritis, multiple serous effusions, eosinophilia and other systemic inflammatory or immune symptoms. The lymphoma cells of angioimmunoblastic T-cell lymphoma originates from follicular helper T cells, and the follicular structure of lymph nodes disappears. In the tumor microenvironment, in addition to tumor cells, there are a large number of over-activated immune cells, such as abnormally activated B cells, which produce a series of systemic inflammation or immune-related symptoms. This disease is rare and difficult to diagnose. This article reports a 36-year-old female. She got fever, joint swelling and pain, skin pigmentation, accompanied by hepatomegaly, splenomegaly, lymphadenopathy, anemia and other multiple-systems manifestations. The clinical manifestations of this patient were similar to autoimmune diseases such as adult onset Still's disease, rheumatoid arthritis, and systemic sclerosis, which made the diagnosis difficult. At the beginning of the disease course, the patient got arthritis and fever. And her white blood cells were significantly increased. Adult onset Still's disease should be considered, but her multiple-systems manifestations could not be explained by adult onset Still's disease. And her arthritis of hands should be distinguished with rheumatoid arthritis. However, the patient's joint swelling could get better within 3-7 days, and there was no synovitis and bone erosion on joint imaging examination. The rheumatoid factor and anti-CCP antibody were negative. The diagnostic evidence for rheumatoid arthritis was insufficient. The patient's skin pigmentation and punctate depigmentation were similar to those of systemic sclerosis. But the patient had no Raynaud's phenomenon, and her sclerosis-related antibody was negative. The diagnostic evidence for systemic sclerosis was also insufficient. After 3 years, she was finally diagnosed with angioimmunoblastic T-cell lymphoma by lymph node biopsy aspiration. This case suggests that the clinical manifestations of angioimmunoblastic T-cell lymphoma are diverse, and some symptoms similar to immune diseases may appear. When the patient's clinical symptoms are atypical and immune diseases cannot explain the patient's condition, and further evidence should be sought to confirm the diagnosis.
血管免疫母细胞性T细胞淋巴瘤是一种罕见的T细胞淋巴瘤。其临床表现不具有特异性。除了淋巴瘤常见的临床表现如发热、体重减轻、盗汗及淋巴结病外，还可能有皮疹、关节炎、多浆膜腔积液、嗜酸性粒细胞增多等全身炎症或免疫症状。血管免疫母细胞性T细胞淋巴瘤的淋巴瘤细胞起源于滤泡辅助性T细胞，淋巴结的滤泡结构消失。在肿瘤微环境中，除肿瘤细胞外，还有大量过度活化的免疫细胞，如异常活化的B细胞，产生一系列全身炎症或免疫相关症状。本病罕见且诊断困难。本文报道一名36岁女性。她出现发热、关节肿胀疼痛、皮肤色素沉着，伴有肝肿大、脾肿大、淋巴结病、贫血等多系统表现。该患者的临床表现与成人斯蒂尔病、类风湿关节炎、系统性硬化症等自身免疫性疾病相似，使得诊断困难。在病程初期，患者有关节炎和发热。且其白细胞显著升高。应考虑成人斯蒂尔病，但成人斯蒂尔病无法解释其多系统表现。其手部关节炎应与类风湿关节炎相鉴别。然而，患者的关节肿胀在3 - 7天内可好转，关节影像学检查未发现滑膜炎及骨质侵蚀。类风湿因子和抗环瓜氨酸肽抗体均为阴性。类风湿关节炎的诊断依据不足。患者的皮肤色素沉着和点状色素脱失与系统性硬化症相似。但患者无雷诺现象，且其硬化相关抗体为阴性。系统性硬化症的诊断依据也不足。3年后，经淋巴结穿刺活检最终诊断为血管免疫母细胞性T细胞淋巴瘤。该病例提示血管免疫母细胞性T细胞淋巴瘤临床表现多样，可能出现一些与免疫性疾病相似的症状。当患者临床症状不典型且免疫性疾病无法解释病情时，应进一步寻找证据以明确诊断。
