Copyright
©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract complicated by protein-losing enteropathy: A case report
Shan Jiang, Ling-Jian Wang, Cong-Wei Jia, Wei Zhang, Wei Wang, Hai-Long Li, Xiao-Hong Sun, Xuan Qu, Lin Kang
Shan Jiang, Xiao-Hong Sun, Xuan Qu, Lin Kang, Department of Geriatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
Ling-Jian Wang, Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
Cong-Wei Jia, Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
Wei Zhang, Wei Wang, Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
Hai-Long Li, Department of Clinical Nutrition, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
Co-first authors: Shan Jiang and Ling-Jian Wang.
Co-corresponding authors: Xuan Qu and Lin Kang.
Author contributions: Jiang S, Wang LJ contributed to manuscript writing, editing, and data collection; Jia CW assisted with the interpretation of pathological specimens; Zhang W and Wang W contributed to the diagnosis and treatment of the disease; Li HL, Qu X, Sun XH, and Kang L provided support during patient follow-up and data collection; Jiang S, Qu X, Kang L contributed to manuscript revision, data collection, conceptualization and supervision. All authors have read and approved the final manuscript. Jiang S and Wang LJ contributed equally to this work as co-first authors. The reason for this designation is based on the complementary and essential contributions made by both authors during the preparation of the manuscript and the conduct of the study. One author (Kang L) was primarily responsible for the conception, design, and coordination of the clinical aspects, while the other (Qu X) led the data interpretation, and manuscript drafting and revisions. Given the interdisciplinary nature of the study and the need for effective communication in clinical contexts, it is appropriate and necessary for both to serve as corresponding authors. This arrangement ensures timely and accurate responses to any queries that may arise during the review and publication process.
Supported by The National High Level Hospital Clinical Research Funding, No. 2022-PUMCH-B-132.
Informed consent statement: Written informed consent was obtained from the patient to publish this paper.
Conflict-of-interest statement: Authors declare that they have no competing interests.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
https://creativecommons.org/Licenses/by-nc/4.0/ Corresponding author: Lin Kang, MD, PhD, Chief Physician, Department of Geriatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, China.
kangl@pumch.cn
Received: March 17, 2025
Revised: April 12, 2025
Accepted: May 13, 2025
Published online: May 28, 2025
Processing time: 71 Days and 0.9 Hours
BACKGROUND
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.
CASE SUMMARY
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.
CONCLUSION
iNKLPD generally exhibits an indolent course; nonetheless, the prognosis may be poor if secondary PLE is involved.
Core Tip: Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract is a rare disease characterized by indolent behavior, for which diagnosis is primarily dependent on histopathological examination. Most cases are managed conservatively with follow-up observation, allowing patients to live with the disease for prolonged periods. Herein, we report the case of a patient with gastrointestinal indolent NK cell lymphoproliferative disorder presenting with protein-losing enteropathy as a prominent manifestation. When this condition arises, the prognosis may be poor.
