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...
Saved in:
Main Authors: | , , , , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Wiley
2016-01-01
|
Series: | Case Reports in Hematology |
Online Access: | http://dx.doi.org/10.1155/2016/9351408 |
Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
_version_ | 1832561260064931840 |
---|---|
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. |
format | Article |
id | doaj-art-fbe7775248c8403586ffabd3b11e0349 |
institution | Kabale University |
issn | 2090-6560 2090-6579 |
language | English |
publishDate | 2016-01-01 |
publisher | Wiley |
record_format | Article |
series | Case Reports in Hematology |
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 |
work_keys_str_mv | AT nadinestanek marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT peterbauerfeind marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT guidoherzog marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT henrietteheinrich marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT matthiassauter marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT danielalenggenhager marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT caciliareiner marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT markusgmanz marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT jeroensgoede marginalzonelymphomacomplicatedbyproteinlosingenteropathy AT benjaminmisselwitz marginalzonelymphomacomplicatedbyproteinlosingenteropathy |