Jiang Shan, Wang Ling-Jian, Jia Cong-Wei, Zhang Wei, Wang Wei, Li Hai-Long, Sun Xiao-Hong, Qu Xuan, Kang Lin
Department of Geriatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
World J Radiol. 2025 May 28;17(5):107141. doi: 10.4329/wjr.v17.i5.107141.
Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract (iNKLPD) is a rare and recently defined entity, recognized in the 2022 WHO classification of hematolymphoid tumors. iNKLPD typically exhibits a benign or slowly progressive clinical course, with disease localized to the gastrointestinal tract. Here, we present what we believe to be the first reported case of iNKLPD associated with protein-losing enteropathy (PLE), characterized by a poor response to chemotherapy and rapid clinical deterioration, culminating in death within a few months.
We report the case of a 64-year-old man who presented with bilateral lower-extremity edema and fatigue. Laboratory tests revealed marked hypoalbuminemia, while other liver function parameters remained within normal limits. Renal and cardiac function assessments were unremarkable. Histopathological examination of endoscopic biopsies confirmed a diagnosis of iNKLPD of the gastrointestinal tract. The patient was treated with oral prednisone and cyclosporine, which led to temporary improvement in both symptoms and serum albumin levels. However, disease relapse occurred during corticosteroid tapering, accompanied by worsening hypoalbuminemia and refractory diarrhea. The patient died eight months after diagnosis, likely due to disease progression or severe treatment-related complications.
iNKLPD generally exhibits an indolent course; nonetheless, the prognosis may be poor if secondary PLE is involved.
胃肠道惰性自然杀伤细胞淋巴增殖性疾病（iNKLPD）是一种罕见且近期才定义的疾病实体，在2022年世界卫生组织血液淋巴系统肿瘤分类中得到认可。iNKLPD通常表现为良性或缓慢进展的临床病程，病变局限于胃肠道。在此，我们报告我们认为是首例与蛋白丢失性肠病（PLE）相关的iNKLPD病例，其特征为对化疗反应不佳且临床迅速恶化，最终在数月内死亡。
我们报告一名64岁男性患者，其出现双侧下肢水肿和乏力。实验室检查显示明显低白蛋白血症，而其他肝功能参数仍在正常范围内。肾脏和心脏功能评估无异常。内镜活检的组织病理学检查确诊为胃肠道iNKLPD。患者接受口服泼尼松和环孢素治疗，症状和血清白蛋白水平均有暂时改善。然而，在糖皮质激素减量过程中疾病复发，伴有低白蛋白血症加重和难治性腹泻。患者在诊断后8个月死亡，可能是由于疾病进展或严重的治疗相关并发症。
iNKLPD通常病程惰性；然而，如果合并继发性PLE，预后可能较差。
