{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T02:58:06Z","timestamp":1789268286010,"version":"build-2803163510"},"reference-count":17,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T00:00:00Z","timestamp":1639180800000},"content-version":"vor","delay-in-days":10,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81800543"],"award-info":[{"award-number":["81800543"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Gastroenterol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Increasing evidence has demonstrated an association between PIL and lymphoma.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 54-year-old man with a 20-year history of abdominal distension and bilateral lower limb edema was admitted. Laboratory investigations revealed lymphopenia, hypoalbuminemia, decreased triglyceride and cholesterol level. Colonoscopy showed multiple smooth pseudo polyps in the ileocecal valve and terminal ileum and histological examination showed conspicuous dilation of the lymphatic channels in the mucosa and submucosa. A diagnosis of PIL was made. Three years later colonoscopy of the patient showed an intraluminal proliferative mass in the ascending colon and biopsy examination confirmed a malignant non-Hodgkin lymphoma. Then the patient was been underwent chemotherapy, and his clinical condition is satisfactory.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Our report supports the hypothesis that PIL is associated with lymphoma development.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12876-021-01997-x","type":"journal-article","created":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T08:02:31Z","timestamp":1639209751000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma"],"prefix":"10.1186","volume":"21","author":[{"given":"Doudou","family":"Hu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xianghua","family":"Cui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wanlei","family":"Ren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xin","family":"Guan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiangjun","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,12,11]]},"reference":[{"key":"1997_CR1","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1186\/1750-1172-3-5","volume":"3","author":"S Vignes","year":"2008","unstructured":"Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann\u2019s disease). Orphanet J Rare Dis. 2008;3:5.","journal-title":"Orphanet J Rare Dis"},{"key":"1997_CR2","doi-asserted-by":"publisher","first-page":"296","DOI":"10.1136\/gut.47.2.296","volume":"47","author":"Y Bouhnik","year":"2000","unstructured":"Bouhnik Y, Etienney I, Nemeth J, Thevenot T, Lavergne-Slove A, Matuchansky C. Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. Gut. 2000;47:296\u2013300.","journal-title":"Gut"},{"key":"1997_CR3","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1016\/S0016-5085(19)35130-3","volume":"41","author":"TA Waldmann","year":"1961","unstructured":"Waldmann TA, Steinfeld JL, Dutcher TF, Davidson JD, Gordon RS Jr. The role of the gastrointestinal system in \u201cidiopathic hypoproteinemia.\u201d Gastroenterology. 1961;41:197\u2013207.","journal-title":"Gastroenterology"},{"key":"1997_CR4","doi-asserted-by":"publisher","first-page":"3466","DOI":"10.1007\/s10620-010-1161-1","volume":"55","author":"J Wen","year":"2010","unstructured":"Wen J, Tang Q, Wu J, Wang Y, Cai W. Primary intestinal lymphangiectasia: four case reports and a review of the literature. Dig Dis Sci. 2010;55:3466\u201372.","journal-title":"Dig Dis Sci"},{"key":"1997_CR5","doi-asserted-by":"publisher","first-page":"1355","DOI":"10.1023\/A:1008496900822","volume":"9","author":"J Gum\u00e0","year":"1998","unstructured":"Gum\u00e0 J, Rubi\u00f3 J, Masip C, Alvaro T, Borr\u00e0s JL. Aggressive bowel lymphoma in a patient with intestinal lymphangiectasia and widespread viral warts. Ann Oncol. 1998;9:1355\u20136.","journal-title":"Ann Oncol"},{"key":"1997_CR6","doi-asserted-by":"publisher","first-page":"512","DOI":"10.12659\/AJCR.899636","volume":"17","author":"MJ Alshikho","year":"2016","unstructured":"Alshikho MJ, Talas JM, Noureldine SI, Zazou S, Addas A, Kurabi H, Nasser M. Intestinal lymphangiectasia: insights on management and literature review. Am J Case Rep. 2016;17:512\u201322.","journal-title":"Am J Case Rep"},{"key":"1997_CR7","doi-asserted-by":"publisher","first-page":"605","DOI":"10.7326\/0003-4819-77-4-605","volume":"77","author":"Immunodeficiency Disease and Malignancy","year":"1972","unstructured":"Immunodeficiency Disease and Malignancy. Various immunologic deficiencies of man and the role of immune processes in the control of malignant disease. Ann Intern Med. 1972;77:605\u201328.","journal-title":"Ann Intern Med"},{"key":"1997_CR8","doi-asserted-by":"publisher","first-page":"753","DOI":"10.1136\/pgmj.53.626.753","volume":"53","author":"M Ward","year":"1977","unstructured":"Ward M, Le Roux A, Small WP, Sircus W. Malignant lymphoma and extensive viral wart formation in a patient with intestinal lymphangiectasia and lymphocyte depletion. Postgrad Med J. 1977;53:753\u20137.","journal-title":"Postgrad Med J"},{"key":"1997_CR9","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1016\/0016-5085(81)90208-0","volume":"80","author":"S Broder","year":"1981","unstructured":"Broder S, Callihan TR, Jaffe ES, DeVita VT, Strober W, Bartter FC, Waldmann TA. Resolution of longstanding protein-losing enteropathy in a patient with intestinal lymphangiectasia after treatment for malignant lymphoma. Gastroenterology. 1981;80:166\u20138.","journal-title":"Gastroenterology"},{"key":"1997_CR10","doi-asserted-by":"publisher","first-page":"512","DOI":"10.1056\/NEJM198402233100808","volume":"310","author":"Case Records of the Massachusetts General Hospital","year":"1984","unstructured":"Case Records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 8\u20131984. An elderly woman with protein-losing enteropathy and pleural effusions. N Engl J Med. 1984;310:512\u201320.","journal-title":"N Engl J Med"},{"key":"1997_CR11","first-page":"299","volume":"27","author":"P Herait","year":"1985","unstructured":"Herait P, Gisselbrecht C, Ferme C, Jian R, Modigliani R, Griscelli C, Hervio P, Marty M, Boiron M. Non-Hodgkin lymphoma arising in a case of Waldmann\u2019s intestinal lymphangiectasis. Nouv Rev Fr Hematol. 1985;27:299\u2013302.","journal-title":"Nouv Rev Fr Hematol"},{"key":"1997_CR12","doi-asserted-by":"publisher","first-page":"147","DOI":"10.3109\/10428199309054743","volume":"11","author":"O Shpilberg","year":"1993","unstructured":"Shpilberg O, Shimon I, Bujanover Y, Ben-Bassat I. Remission of malabsorption in congenital intestinal lymphangiectasia following chemotherapy for lymphoma. Leuk Lymphoma. 1993;11:147\u20138.","journal-title":"Leuk Lymphoma"},{"key":"1997_CR13","doi-asserted-by":"publisher","first-page":"1417","DOI":"10.1097\/00042737-200512000-00024","volume":"17","author":"D Laharie","year":"2005","unstructured":"Laharie D, Degenne V, Laharie H, Cazorla S, Belleannee G, Couzigou P, Amouretti M. Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia. Eur J Gastroenterol Hepatol. 2005;17:1417\u20139.","journal-title":"Eur J Gastroenterol Hepatol"},{"key":"1997_CR14","doi-asserted-by":"publisher","first-page":"458","DOI":"10.1159\/000499450","volume":"37","author":"D Prasad","year":"2019","unstructured":"Prasad D, Srivastava A, Tambe A, Yachha SK, Sarma MS, Poddar U. Clinical profile, response to therapy, and outcome of children with primary intestinal lymphangiectasia. Dig Dis. 2019;37:458\u201366.","journal-title":"Dig Dis"},{"key":"1997_CR15","doi-asserted-by":"publisher","first-page":"1643","DOI":"10.1172\/JCI105656","volume":"46","author":"W Strober","year":"1967","unstructured":"Strober W, Wochner RD, Carbone PP, Yachha SK, Sarma MS, Poddar U. Intestinal lymphangiectasia: a protein-losing enteropathy with hypogammaglobulinemia, lymphocytopenia and impaired homograft rejection. J Clin Investig. 1967;46:1643\u201356.","journal-title":"J Clin Investig"},{"key":"1997_CR16","doi-asserted-by":"publisher","first-page":"1319","DOI":"10.1172\/JCI106928","volume":"51","author":"PL Weiden","year":"1972","unstructured":"Weiden PL, Blaese RM, Strober W, Block JB, Waldmann TA. Impaired lymphocyte transformation in intestinal lymphangiectasia: evidence for at least two functionally distinct lymphocyte populations in man. J Clin Investig. 1972;51:1319\u201325.","journal-title":"J Clin Investig"},{"key":"1997_CR17","doi-asserted-by":"publisher","first-page":"605","DOI":"10.1056\/NEJM197903153001106","volume":"300","author":"TA Fleisher","year":"1979","unstructured":"Fleisher TA, Strober W, Muchmore AV, Broder S, Krawitt EL, Waldmann TA. Corticosteroid-responsive intestinal lymphangiectasia secondary to an inflammatory process. N Engl J Med. 1979;300:605\u20136.","journal-title":"N Engl J Med"}],"container-title":["BMC Gastroenterology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12876-021-01997-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12876-021-01997-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12876-021-01997-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T20:05:55Z","timestamp":1639253155000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcgastroenterol.biomedcentral.com\/articles\/10.1186\/s12876-021-01997-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":17,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["1997"],"URL":"https:\/\/doi.org\/10.1186\/s12876-021-01997-x","relation":{},"ISSN":["1471-230X"],"issn-type":[{"value":"1471-230X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,12]]},"assertion":[{"value":"6 January 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 October 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 December 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The study was approved by the Institutional Ethics Committee of Qingdao Municipal Hospital. Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"461"}}