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.

Figure 1

Small bowel (a) and large bowel (b) biopsy samples with minimal ectasia of lymphatic vessels (arrowheads) without other histopathologic findings (H&E). Bone marrow biopsy (c and d) with monotonous infiltrates of a low-grade non-Hodgkin lymphoma (c; H&E), highlighted by immunohistochemistry (d; B cell marker CD79a) (arrowheads). Scale bars = 100 µm.

Figure 2

Evolution of representative disease markers. Courses of Karnofsky index, body weight, serum albumin, bone marrow infiltration, and timing of treatment are indicated. Please note different units of all markers (% for Karnofsky index and bone marrow infiltration, kg for body weight, and g/L for serum albumin). RTX: rituximab; benda: bendamustine.