Wu Miao-Miao, Fu Wen-Jun, Wu Jia, Zhu Lin-Lin, Niu Ting, Yang Rong, Yao Jin, Lu Qiang, Liao Xiao-Yang
General Practice Ward/International Medical Center Ward, General Practice Medical Center, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
Department of Haematology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
World J Clin Cases. 2022 Sep 16;10(26):9417-9427. doi: 10.12998/wjcc.v10.i26.9417.
Peripheral T-cell lymphoma (PTCL), an aggressive and rare disease that belongs to a heterogeneous group of mature T-cell lymphomas, develops rapidly and has a poor prognosis. Early detection and treatment are essential to improve patient cure and survival rates. Here, we report a rare case of PTCL with clinical presentation of noncirrhotic portal hypertension, which provides a basis for early vigilance of lymphomas in the future.
A 65-year-old Chinese woman was admitted to our hospital because of abdominal distension for 3 months and pitting oedema of both lower limbs for 2 months. Physical examinations and associated auxiliary examinations showed the presence of hepatosplenomegaly, and her hepatic venous pressure gradient was 10 mmHg. Immunohistochemical analysis of the liver biopsy confirmed the diagnosis of PTCL. The patient underwent combination therapy with dexamethasone, VP-16, and chidamide. Unfortunately, after 41 days of chemotherapy, the patient died of multiple organ failure.
PCTL accompanied by noncirrhotic portal hypertension is rarely reported. This case report discusses the diagnosis of a patient according to the literature.
外周T细胞淋巴瘤(PTCL)是一种侵袭性罕见疾病,属于成熟T细胞淋巴瘤的异质性群体,发展迅速且预后较差。早期检测和治疗对于提高患者治愈率和生存率至关重要。在此,我们报告一例罕见的PTCL病例,其临床表现为非肝硬化性门静脉高压,为未来早期警惕淋巴瘤提供了依据。
一名65岁中国女性因腹胀3个月、双下肢凹陷性水肿2个月入院。体格检查及相关辅助检查显示肝脾肿大,肝静脉压力梯度为10 mmHg。肝脏活检的免疫组织化学分析确诊为PTCL。患者接受了地塞米松、VP-16和西达本胺的联合治疗。不幸的是,化疗41天后,患者死于多器官功能衰竭。
伴有非肝硬化性门静脉高压的PCTL鲜有报道。本病例报告根据文献对一名患者的诊断进行了讨论。