Prognosis and predictive factors

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The clinical course is very unfavorable due to complications from peritonitis and malnutrition and later from progressive disease typically characterized by intestinal recurrences. The malabsorption due to underlying coeliac disease is detrimental to these patients, particularly when recovering from surgery or receiving multiagent chemotherapy {444}. Consequently, only one half of the patients is amenable to chemotherapy

Fig. 4.24 CD3 immunoexpression in a T-cell lymphoma of the small intestine.

and only a proportion of these is able to finish the complete course. The overall median survival in the largest published series is only 3 months, and 5-year survival in this and other series ranges from

8-25% {305, 424, 444}. The small group of long-term survivors usually received chemotherapy and, interestingly, none had a previous diagnosis of coeliac disease {305, 444}.

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