Enteropathy-associated T-cell lymphoma

A rare type of T-cell lymphoma associated with celiac disease

Complication of coeliac disease

Enteropathy-associated T-cell lymphoma (formerly termed enteropathy-associated T-cell lymphoma, type 1)
Synonyms	Enteropathy-associated T-cell lymphoma, type I
Pronounce
Field	Oncology, hematology, gastroenterology
Symptoms	Weight loss, diarrhea, abdominal pain, nausea, vomiting, fatigue, fever, malabsorption, and gastrointestinal bleeding
Complications	Bowel obstructions, bowel perforations, intestinal perforation, secondary infections due to immunosuppression
Onset	Typically occurs after long-term, untreated Celiac disease
Duration	Variable, can range from months to years depending on early detection and treatment
Types	Primarily classified into two subtypes: Type I (typical) and Type II (atypical)
Causes	Complication of Celiac disease
Risks	Genetic predisposition, long-standing untreated celiac disease, immunosuppression, chronic intestinal inflammation
Diagnosis	Diagnosis is confirmed by histological examination of biopsy specimens, usually from the small intestine. Immunohistochemistry is used to identify the characteristic T-cell markers.
Differential diagnosis	Chronic inflammatory bowel disease, Crohn's disease, intestinal lymphoma, malabsorption syndromes
Prevention	Gluten-free diet, early detection and management of celiac disease, continuous monitoring of individuals with celiac disease for signs of lymphoma
Treatment	Chemotherapy, immunotherapy, radiation therapy, bone marrow transplantation in advanced cases, and a strict gluten-free diet to prevent disease progression
Medication	Chemotherapy agents such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), targeted therapy (e.g., alemtuzumab), and other immunosuppressive treatments may be used.
Prognosis	Guarded, with poor outcomes in advanced stages, but can improve with early detection and treatment. The prognosis is significantly improved with a strict gluten-free diet.
Frequency	Rare, approximately 1-2 cases per 1 million people annually
Deaths	High mortality rate in advanced stages due to complications such as infection, bowel perforation, or progression to other types of lymphoma

Enteropathy-associated T-cell lymphoma (EATL) is a rare type of non-Hodgkin lymphoma that originates from T-cells and is associated with celiac disease. It primarily affects the small intestine and is characterized by the proliferation of malignant T-cells in the intestinal lining.

Pathophysiology

EATL is closely linked to celiac disease, an autoimmune disorder triggered by the ingestion of gluten. In individuals with celiac disease, chronic inflammation of the small intestine can lead to the development of EATL. The persistent immune response against gluten results in damage to the intestinal mucosa, which may eventually lead to the transformation of normal T-cells into malignant ones.

There are two types of EATL:

• Type I EATL: This is the classic form associated with celiac disease. It is more common in individuals of Northern European descent and is characterized by the presence of large, pleomorphic T-cells.
• Type II EATL: Also known as monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), this form is not associated with celiac disease and can occur in individuals without gluten sensitivity. It is characterized by monomorphic, small to medium-sized T-cells.

Clinical Presentation

Patients with EATL often present with non-specific symptoms, which can include:

• Abdominal pain
• Weight loss
• Diarrhea
• Intestinal obstruction
• Perforation of the intestine

Due to its association with celiac disease, patients may also exhibit symptoms related to gluten sensitivity, such as malabsorption and nutritional deficiencies.

Diagnosis

The diagnosis of EATL involves a combination of clinical evaluation, imaging studies, and histopathological examination. Key diagnostic steps include:

• Endoscopy and Biopsy: Endoscopic examination of the small intestine with biopsy is crucial for identifying the characteristic histological features of EATL.
• Imaging: CT scans and MRI may be used to assess the extent of the disease and identify complications such as intestinal obstruction or perforation.
• Histopathology: Examination of biopsy samples reveals atypical T-cells infiltrating the intestinal mucosa. Immunohistochemical staining is used to confirm the T-cell origin of the lymphoma.

Treatment

The treatment of EATL is challenging due to its aggressive nature and the poor overall prognosis. Treatment options may include:

• Chemotherapy: Combination chemotherapy regimens are commonly used, although the response rates are variable.
• Surgery: Surgical intervention may be necessary to manage complications such as intestinal obstruction or perforation.
• Stem Cell Transplantation: In some cases, high-dose chemotherapy followed by autologous stem cell transplantation may be considered.

Prognosis

The prognosis for patients with EATL is generally poor, with a median survival of less than two years. Factors influencing prognosis include the stage of the disease at diagnosis, the patient's overall health, and the response to treatment.

Related Pages

• Celiac disease
• Non-Hodgkin lymphoma
• T-cell
• Small intestine

External links

Classification	ICD-10: C86.2
External resources	Orphanet: 86880

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