Protein-losing enteropathy as a rare presentation of small bowel T-cell post-transplant lymphoproliferative disease

Posttransplant lymphoproliferative disorders (PTLD) represent a heterogeneous group of lymphoproliferative lesions in patients who have solid organ or bone marrow transplantation. While majority of PTLD are of B-cell origin, T-cell PTLD is extremely rare. Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare primary intestinal lymphoma which most commonly involves the small bowel. Since the symptoms at presentation are often vague, the diagnosis is frequently delayed until acute complications such as intestinal perforation or obstruction occur. Here we report a very rare case of posttransplant small bowel MEITL in a kidney transplant recipient presenting with protein-losing enteropathy (PLE) as the initial manifestation. Our patient initially presented with episodic diarrhea but early investigations failed to identify any bowel pathology. Subsequently she developed hypoalbuminemia-related fluid retention and Tc-99 m HSA scintigraphy confirmed the presence of PLE. Even though the upper gastrointestinal endoscopy, colonoscopy and abdominal computed tomography did not show any significant lesions, the patient developed sudden bowel perforation and histologic examination of the resected tissue confirmed the diagnosis of posttransplant MEITL. She developed severe sepsis postoperatively and finally passed away despite aggressive supportive treatment. Our case shows that early diagnosis and prompt treatment is critical to potentially improve the patient outcomes.