2A90.7/C86.2 Enteropathy associated T-cell lymphoma



Enteropathy-associated T-cell lymphoma (EATL) is an aggressive T-cell lymphoma associated with a chronic intestinal disorder such as celiac disease, Crohn’s disease, tropical sprue, or other enteropathies. It is rare, with an estimated annual incidence of less than 1 case per million population and is more common in people of Northern European ancestry. The exact cause of EATL is unknown, although it is thought to be caused by a combination of genetic, environmental and immunological factors.


Diagnosis of EATL is based on a combination of clinical, laboratory and histological findings. Clinical findings include weight loss, abdominal pain, diarrhoea, anaemia and fever. Laboratory findings may include elevated white blood cell count, low albumin, elevated lactate dehydrogenase, and elevated serum alpha-fetoprotein. Histopathological examination of biopsy tissue reveals infiltration of the small intestine by atypical lymphoid cells.

Differential diagnosis

Differential diagnoses of EATL include other T-cell lymphomas, such as peripheral T-cell lymphoma, unspecified, as well as other disorders that can cause similar clinical and laboratory findings, such as Crohn’s disease, ulcerative colitis, and other inflammatory bowel diseases.


Treatment of EATL typically involves a combination of chemotherapy and radiotherapy. In some cases, stem cell transplantation may be recommended.


The prognosis for EATL is generally poor, with a 5-year survival rate of approximately 30%. Survival rates vary depending on the stage of the disease and the patient’s overall health.

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