A rare case of enteropathy-associated T-cell lymphoma presenting as acute renal failure

Abstract

Enteropathy-associated T-cell lymphoma (EATCL) is a high grade, pleomorphic peripheral T-cell lymphoma usually with cytotoxic phenotypes. We describe a first case of patient with EATCL that is remarkable for its fulminant course and invasion of both kidneys manifested as acute renal failure. The patient was a 23 year old woman with a long history of celiac disease. She was presented with acute renal failure and enlarged mononuclear infiltrated kidneys. Diagnosis of tubulointerstitial nephritis and polyserositis was confirmed with consecutive pulse doses of steroid therapy. After recovery, she had disseminated disease two months later. Magnetic resonance imaging showed thickened intestine wall, extremely augmented kidneys, enlarged intra-abdominal lymph nodes with extra-luminal compression of common bile duct. Laparotomy with mesenterial adipous tissue and lymph glands biopsy was done. Consecutive pathophysiological and immunohistochemical analyses confirmed the diagnosis of EATCL: CD45RO+, CD43+, CD3+. The revision of renal pathophysiology substantiated the diagnosis. The patient received chemotherapy, but unfortunately she died manifesting signs of pulmonary embolism caused by tumor cells.

Figure 1

Magnetic resonance imaging shows extremely augmented kidneys.

Figure 2

Neoplastic mesenterial lymph node infiltration with staining of lymphoma cells (APAAP with anti-CD3 Ab x 400).

Figure 3

Renal infiltration with monomorphous lymphoid infiltration (H&E, x 40).

Figure 4

Neoplastic lymphoid cells in ascites (MGG, x100).

Figure 5

IgA antiendomysial antibodies surrounding sarcolemma of smoth muscle fibers in lamina muscularis mucosae of monkey esophagus (IIF, x 400).