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Case Reports in Hematology
Volume 2016, Article ID 9351408, 5 pages
http://dx.doi.org/10.1155/2016/9351408
Case Report

Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy

1Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland
2Department of Gastroenterology, Stadtspital Triemli Zurich and Zurich University, Zurich, Switzerland
3Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich University, Zurich, Switzerland
4Department of Radiology, University Hospital Zurich, Zurich University, Zurich, Switzerland
5Department of Hematology, University Hospital Zurich, Zurich University, Zurich, Switzerland

Received 4 August 2016; Accepted 13 October 2016

Academic Editor: Salah Aref

Copyright © 2016 Nadine Stanek et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Protein losing enteropathy (PLE) refers to excessive intestinal protein loss, resulting in hypoalbuminemia. Underlying pathologies include conditions leading to either reduced intestinal barrier or lymphatic congestion. We describe the case of a patient with long-lasting diffuse abdominal problems and PLE. Repetitive endoscopies were normal with only minimal lymphangiectasia in biopsies. Further evaluations revealed an indolent marginal zone lymphoma with minor bone marrow infiltration. Monotherapy with rituximab decreased bone marrow infiltration of the lymphoma but did not relieve PLE. Additional treatments with steroids, octreotide, a diet devoid of long-chain fatty-acids, and parenteral nutrition did not prevent further clinical deterioration with marked weight loss (23 kg), further reduction in albumin concentrations (nadir 8 g/L), and a pronounced drop in performance status. Finally, immunochemotherapy with rituximab and bendamustine resulted in hematological remission and remarkable clinical improvement. 18 months after therapy the patient remains free of gastrointestinal complaints and has regained his body weight with normal albumin levels. We demonstrate a case of PLE secondary to indolent marginal zone lymphoma. No intestinal pathologies were detected, contrasting a severe and almost lethal clinical course. Immunochemotherapy relieved lymphoma and PLE, suggesting that a high suspicion of lymphoma is warranted in otherwise unexplained cases of PLE.