IDDF2018-ABS-0169 Clinical characteristics of enteropathy-associated t-cell lymphoma (EATL) with intracranial metastasis: an analysis of published case reports

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Abstract

Background

To describe the clinical characteristics of EATL with intracranial metastasis from cases published in literature

Subjects

EATL is a rare disease of the gastrointestinal tract. Intracranial metastasis associated with EATL is even rare. Six cases including one of ours were recruited in the study. Five reported cases were found between 1997 and 2012 in the English literature (table 1).

Methods

Ours was a 35-year-old man who presented initially with diarrhoea, and he was later confirmed to have EATL from biopsy at jejunum that showing positive in CD3, CD8 and CD56 with negative in CD4. Since diagnosis, he had received 11 cycles of adjuvant chemotherapy. He underwent laparotomy with resection and anastomosis of jejunum due to the acute abdomen. An episode of left-sided weakness after having received his 11th cycle of chemotherapy led to brain computed tomography scan, which it showed brain metastasis. He expired 9 months later after initial diagnosis. Altogether, six cases of EATL with intracranial metastasis were reviewed for their clinical characteristics.

Results

The mean age was 54 years old (range from 35 to 65 years old). Two-thirds were males. Many (two-thirds) were without a history of celiac disease. One-half of the patients had abdominal pain as the initial gastrointestinal presentation. The primary lesion site was in the small intestine with jejunum predominance (66.6%). Eighty-three percent of patients had abdominal surgery and adjuvant chemotherapy for the primary lesion. The majority site of intracranial metastasis was supratentorial V.S. infratentorial; 83% V.S 17%. Half of intracranial metastasis (50%) presented with a change of mental status. Headache (33.3%) and weakness of extremities (33.3%) were the next most common presentations of metastasis. Radiotherapy, chemotherapy and steroid therapy were used to treat intracranial metastasis. The survival was an average of 9.6 months (3 months to 16 months) after the initial diagnosis of EATL.

Conclusions

The prognosis of EATL with intracranial metastasis is generally poor with a mean survival time of fewer than 10 months.

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