Enteropathy-associated T-cell lymphoma

Orpha code: 86880OMIM code:

Definition

A rare T-cell non-Hodgkin lymphoma characterized by a neoplasm of intraepithelial T-cells mostly occurring in the jejunum or ileum in patients with celiac disease. The lesion may be multifocal and form ulcerating nodules, plaques, strictures, or an exophytic mass. The mesentery and mesenteric lymph nodes are commonly involved. Patients typically present with abdominal pain, malabsorption or diarrhea, anorexia, weight loss, fatigue, nausea, vomiting, and sometimes intestinal perforation or hemorrhage. Prognosis is generally poor.

Disease data
Classification

Disease

Synonyms
EATL
EATL
Enteropathy-type T-cell lymphoma
ETTL
Jelitowy chłoniak z komórek T
ETTL
Enteropathy-type T-cell lymphoma
Intestinal T-cell lymphoma
ORPHA code
86880
OMIM code
-
ICD10 code
C86.2
ICD11 code
2A90.7

No additional description.

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