The story of the lost proteins

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S.E. van der Wiel, A.G.L. Bodelier, J.W.J. van Esser

Voorzitter(s): prof. dr. M.M.E. Schneider, UMCU, Utrecht & dr. L.J.M. de Heide, Zorgroep Noorderbreedt, Leeuwarden

Woensdag 22 april 2015

15:00 - 16:00u in Zaal 0.4

Categorieën: parallelsessie (case reports/research)

Parallel sessie: Parallelsessie 3: Case reports/research

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Introduction:
Protein-losing enteropathy (PLE) can be a manifestation of several disorders, characterized by excessive loss of plasma proteins into the gastrointestinal tract. Treatment of underlying disease will mostly solve the protein loss, however not in our case.

Case:
A 69- year old male with no relevant medical history, was admitted to our hospital with complaints of fatigue and bilateral peripheral oedema. Physical examination was unremarkable, except tibial oedema. Laboratory testing revealed a decreased serum albumin (25 g/L), total protein (41 g/L) and elevated faecal Alpha 1-Antitrypsin (576mg/100 gram). Extensive diagnostic evaluation showed no abnormalities. Capsule endoscopy and double balloon enteroscopy was performed, which showed a diffuse whitish appearance of the small bowel mucosa with extensive lymphangiectasia and macroscopic loss of milky fluids. Based on these findings PLE due to extensive small intestinal lymphangiectasia of unknown cause was suspected. A medium chain triglycerides diet was started, later combined with Lanreotide. The oedema resolved and serum albumin and total protein increased, but did not return normal. 

1.5 years after presentation our patient developed abdominal pain with a palpable mass, histological examination showed a diffuse large B-cell Non Hodgkin Lymphoma. Treatment with chemotherapy and Rituximab was initiated, resulting in complete remission. We suspected that the PLE was a paraneoplastic phenomenon, however treatment did not completely resolve symptoms.

Conclusion:
We described a patient with PLE most likely based on Non Hodgkin lymphoma. Despite complete remission, the PLE did not disappear, suggesting the possibility of irreversible damage to the lymphatic system of the small intestine.