Enteropathy-associated T-cell lymphoma

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Abstract

Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumour of intraepithelial T lymphocytes, usually presenting as a neoplasm composed of large lymphoid cells and often associated with necrosis and an inflammatory background, including large numbers of histiocytes and eosinophils. Intestinal intraepithelial α–β T-cells have been postulated as the normal-cell counterpart for EATL. EATL is the most common neoplastic complication of coeliac disease. The disease is uncommon in most parts of the world, but is seen with greater frequency in those areas with a high prevalence of coeliac disease, in particular Northern Europe. Usually, EATL occurs in adults, and generally present with abdominal pain, often associated with jejunal perforation, weight loss, diarrhoea, or bowel obstruction. EATL is characterized by multifocal presentation in 10–25% of cases. Small-bowel lymphoma is more common than large-bowel or rectal lymphomas. The prognosis of EATL is very poor, with low chemosensitivity, rapid tumour growth and a tendency to dissemination. Moreover, the high incidence of severe postsurgical complications and the poor nutritional and immunological conditions lead to progressive deterioration of these patients, preventing the use of an adequate and effective treatment.

Section snippets

Definition

Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumour of intraepithelial T lymphocytes, usually presenting as a neoplasm composed of large lymphoid cells and often associated with necrosis and an inflammatory background, including large numbers of histiocytes and eosinophils. The adjacent small intestinal mucosa shows villous atrophy, crypt hyperplasia, increased lamina propria lymphocytes and plasma cells, and intraepithelial lymphocytosis. In 10–20% of cases, the lymphoma is

Morphology

EATL more often occurs in the jejunum or ileum in the form of one or more ulcerating mucosal lesions that invade the wall of the intestine and frequently cause perforation. This is in contrast to what seen in B-cell lymphomas that tend to affect the distal or terminal ileum by producing annular infiltration or polypoid masses [14], [15]. Classical EATL shows a wide range of cytological appearances [16], [17]. Most commonly, neoplastic cells are rather monotonous, medium-large sized with round

Clinical presentations

Usually, EATL occurs in adults, often with a history of gluten-sensitive enteropathy, but occasionally as the initial event in a patient found to have the typical histological features of sprue in the resected intestine. Less commonly, it arises in patients without evidence of enteropathy; in these cases, diagnosis is difficult and delayed due to the non-specific nature of the symptoms and a very low index of clinical suspicion. Patients generally present with abdominal pain, often associated

Staging procedures

Complete staging work-up for EATL includes an accurate physical examination (Waldeyer's ring involvement should be excluded), complete haematological and biochemical exams, total-body computerized tomography, gastrointestinal tract examination, and bone marrow aspirate and biopsy. Unlike primary gastric lymphoma, where a surgical approach is progressively being replaced by conservative management, most patients with EATL still undergo exploratory laparotomy for diagnosis and staging. In

Natural history

EATL is an aggressive malignancy which, if untreated, leads invariably to death due to multifocal intestinal perforation caused by refractory malignant ulcers. Since its association with gluten-sensitive enteropathy, most patients with EATL are extremely compromised from an immunological and nutritional point of view. Most patients with EATL are managed with a surgical approach as the primary strategy. Even if surgical operation is not a curative treatment, debulking and resection of masses

First-line treatment

A standard treatment for patients with EATL has not been established, and overall reported results with varied modalities are unsatisfactory. The role of surgery is limited to debulking or resection of masses with high-risk of obstruction or perforation and is suitable for individual clinical use on a type R basis. Radiation therapy has been indicated in some patients presenting with bulky disease, rectal lymphoma or incomplete resection. Involved-field delivering 35 Gy in 1.5–2-Gy daily

Conflict of interest

Authors have no conflict of interest to be disclosed.

Reviewers

Julian Malicki, Ph.D., School of Health Sciences, University of Medical Sciences, Great Poland Cancer Centre, Garbary Street 15, PL-61-866 Poznan, Poland.

Andrés J.M. Ferreri is Coordinator of the Unit of Lymphoid Malignancies and Vice Director of the Medical Oncology Unit, Department of Oncology, San Raffaele H Scientific Institute, Milan, Italy.

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    • Cited by (0)

    Andrés J.M. Ferreri is Coordinator of the Unit of Lymphoid Malignancies and Vice Director of the Medical Oncology Unit, Department of Oncology, San Raffaele H Scientific Institute, Milan, Italy.

    Pier Luigi Zinzani is Coordinator of the Lymphoma Unit of the Institute of Hematology “L. e A. Seràgnoli”, University of Bologna, Italy.

    Silvia Govi is Assistant Physician at Unit of Lymphoid Malignancies, Medical Oncology Unit, Department of Oncology, San Raffaele H Scientific Institute, Milan, Italy.

    Stefano A. Pileri is Full Professor of Pathologic Anatomy, Director of the Service of Haematopathology at Bologna University School of Medicine. Director of the Research Doctorate Project “Clinical and Experimental Haematology and Haematopathology”.

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