doiSerbia

Enteropathy associated T-cell lymphoma

Bakrač Milena (Institut za hematologiju, Klinički centar Srbije, Beograd)
Bonači-Nikolić Branka (Institut za alergologiju i imunologiju, Klinički centar Srbije, Beograd)
Čolović Nataša (Institut za hematologiju, Klinički centar Srbije, Beograd)
Simić-Ogrizović Sanja (Institut za nefrologiju, Klinički centar Srbije, Beograd)
Krstić Miodrag (Klinika za gastroenterologiju, Institut za bolesti digestivnog sistema, Klinički centar Srbije, Beograd)
Čolović Milica (Institut za hematologiju, Klinički centar Srbije, Beograd)

Enteropathy associated T-cell lymphoma (EATCL) is a high grade, pleomorphic peripheral T-cell lymphoma with usually cytotoxic phenotype. This is a case report of three patients with EATCL. The first patient was 50 year-old woman with four year history of gluten sensitive enteropathy (GSE). Diagnosis of lymphoma was confirmed after the resection of the jejunum (small intestine obstruction). Pathohistological (PAS, Reticulin, Giemsa) and immunohistochemical (anti-LCA, anti-CD20, anti- CD45RO, anti-CD3) methods revealed the diagnosis of EATCL: CD45RO+, CD3+. After the third cycle of chemotherapy, the disease progressed with massive lung infiltration. Patient died due to complications of bone marrow aplasia. The second patient was 23 year-old woman with long earlier history of GSE. She presented with the acute renal failure. According to established diagnosis of tubulointerstitial nephritis, she was treated with pulse doses of steroid therapy. After temporary improvement, she had dissemination of the disease. On MRI, small intestinal wall was thickened, and abdominal lymph nodes were enlarged with extraluminal compression of common bile duct. Laparotomy with mesenterial lymph node biopsy and consecutive pathohistological and immunohistochemical analyses revealed the diagnosis of EATCL. The patient received chemotherapy, but she died with signs of pulmonary embolization. The third patient was 53 year-old woman without previous history of GSE. Diagnosis of EATCL was revealed after the resection of jejunum because of small intestinal obstruction. She received two cycles of chemotherapy, but she died with signs of disease progression. IgA antiendomysial antibodies were detected in the serum of all patients. The overall survival of patients was 7 months. The possibility of lymphoma rising in patients with clinical progression of GSE despite gluten free diet must be kept in mind.

Keywords: enteropathy associated T-cell lymphoma, glutensensitive enteropathy, anti-endomysial antibodies, clinical features, prognosis