title: Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy.
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.
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Nadine
authors.initials: N
authors.lastname: Stanek
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Peter
authors.initials: P
authors.lastname: Bauerfeind
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Guido
authors.initials: G
authors.lastname: Herzog
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Henriette
authors.initials: H
authors.lastname: Heinrich
authors.affiliation: Department of Gastroenterology, Stadtspital Triemli Zurich and Zurich University, Zurich, Switzerland.
authors.forename: Matthias
authors.initials: M
authors.lastname: Sauter
authors.affiliation: Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Daniela
authors.initials: D
authors.lastname: Lenggenhager
authors.affiliation: Department of Radiology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Cäcilia
authors.initials: C
authors.lastname: Reiner
authors.affiliation: Department of Hematology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Markus G
authors.initials: MG
authors.lastname: Manz
authors.affiliation: Department of Hematology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Jeroen S
authors.initials: JS
authors.lastname: Goede
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
authors.forename: Benjamin
authors.identifier: 0000-0002-8719-5175
authors.initials: B
authors.lastname: Misselwitz