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Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy
Nadine Stanek1, Peter Bauerfeind1, Guido Herzog1
1Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
Case Reports in Hematology
|November 29, 2016
Summary
Protein losing enteropathy (PLE) is excessive intestinal protein loss. This case highlights indolent marginal zone lymphoma as a cause of severe PLE, effectively treated with immunochemotherapy.
Area of Science:
- Gastroenterology
- Hematology
- Oncology
Background:
- Protein losing enteropathy (PLE) involves excessive intestinal protein loss, leading to hypoalbuminemia.
- Pathologies causing PLE include impaired intestinal barrier function or lymphatic congestion.
- Indolent lymphomas can present with gastrointestinal symptoms, including PLE.
Purpose of the Study:
- To report a case of severe protein losing enteropathy secondary to indolent marginal zone lymphoma.
- To illustrate the diagnostic challenges and treatment outcomes in such cases.
- To emphasize the importance of considering lymphoma in unexplained PLE.
Main Methods:
- Case report of a patient with long-standing abdominal issues and PLE.
- Diagnostic workup including endoscopy, biopsies, and bone marrow evaluation.
- Treatment course involving rituximab monotherapy, supportive care, and subsequent immunochemotherapy with rituximab and bendamustine.
Main Results:
- Initial investigations revealed minimal lymphangiectasia, with subsequent diagnosis of indolent marginal zone lymphoma.
- Rituximab monotherapy failed to resolve PLE, despite reducing lymphoma infiltration.
- Immunochemotherapy led to hematological remission and significant clinical improvement, resolving PLE and associated symptoms.
Conclusions:
- Indolent marginal zone lymphoma can cause severe, life-threatening protein losing enteropathy.
- Diagnosis can be challenging due to non-specific endoscopic findings.
- Aggressive immunochemotherapy is effective in treating both the lymphoma and the associated PLE.
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