Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy

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 a...

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Main Authors: Nadine Stanek, Peter Bauerfeind, Guido Herzog, Henriette Heinrich, Matthias Sauter, Daniela Lenggenhager, Cäcilia Reiner, Markus G. Manz, Jeroen S. Goede, Benjamin Misselwitz
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2016/9351408
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author Nadine Stanek
Peter Bauerfeind
Guido Herzog
Henriette Heinrich
Matthias Sauter
Daniela Lenggenhager
Cäcilia Reiner
Markus G. Manz
Jeroen S. Goede
Benjamin Misselwitz
author_facet Nadine Stanek
Peter Bauerfeind
Guido Herzog
Henriette Heinrich
Matthias Sauter
Daniela Lenggenhager
Cäcilia Reiner
Markus G. Manz
Jeroen S. Goede
Benjamin Misselwitz
author_sort Nadine Stanek
collection DOAJ
description 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.
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spelling doaj-art-fbe7775248c8403586ffabd3b11e03492025-02-03T01:25:31ZengWileyCase Reports in Hematology2090-65602090-65792016-01-01201610.1155/2016/93514089351408Marginal Zone Lymphoma Complicated by Protein Losing EnteropathyNadine Stanek0Peter Bauerfeind1Guido Herzog2Henriette Heinrich3Matthias Sauter4Daniela Lenggenhager5Cäcilia Reiner6Markus G. Manz7Jeroen S. Goede8Benjamin Misselwitz9Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Gastroenterology, Stadtspital Triemli Zurich and Zurich University, Zurich, SwitzerlandDepartment of Pathology and Molecular Pathology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Radiology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Hematology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Hematology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandDepartment of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, SwitzerlandProtein 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.http://dx.doi.org/10.1155/2016/9351408
spellingShingle Nadine Stanek
Peter Bauerfeind
Guido Herzog
Henriette Heinrich
Matthias Sauter
Daniela Lenggenhager
Cäcilia Reiner
Markus G. Manz
Jeroen S. Goede
Benjamin Misselwitz
Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy
Case Reports in Hematology
title Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy
title_full Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy
title_fullStr Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy
title_full_unstemmed Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy
title_short Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy
title_sort marginal zone lymphoma complicated by protein losing enteropathy
url http://dx.doi.org/10.1155/2016/9351408
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