Small Intestinal Enteropathy-Associated T-Cell Lymphoma

Preferred Label : Small Intestinal Enteropathy-Associated T-Cell Lymphoma;

NCIt synonyms : Small Intestinal Enteropathy-Type T-Cell Lymphoma; Small Intestinal EATL;

NCIt definition : An enteropathy-associated T-cell lymphoma arising from the small intestine, most commonly the jejunum or ileum. Patients usually present with abdominal pain, often associated with intestinal perforation. It is associated with celiac disease. The lymphoma cells are usually medium-sized to large and form an ulcerating mucosal lesion with invasion of the small intestinal wall. Villous atrophy is present in the adjacent small intestinal mucosa. In a minority of cases the lymphoma cells are medium-sized and form a monomorphic infiltrate.;

Neoplastic status : Malignant;

Details

Origin ID

C39610;

UMLS CUI

C1519371;

Disease excludes abnormal cell
- Neoplastic B-Lymphocyte [NCIt concept]
- Neoplastic Small to Medium-Sized T-Lymphocyte [NCIt concept]
- Reed-Sternberg Cell [NCIt concept]
Disease excludes normal cell origin
- Myeloid Cell [NCIt concept]
- Plasma Cell [NCIt concept]
- Precursor Natural Killer Cell [NCIt concept]
- Precursor T-Lymphoblast [NCIt concept]
Disease may have findings
- Abdominal Pain [NCIt concept]
- Aggressive Clinical Course [NCIt concept]
- Chronic Inflammatory Infiltrate [NCIt concept]
- Intestinal Perforation [NCIt concept]
- Intestinal Ulcerated Mass [NCIt concept]
Has associated anatomic sites
- Digestive System [NCIt concept]
- Hematopoietic and Lymphatic System [NCIt concept]
- Intestine [NCIt concept]
- Small Intestine [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Monomorphic Cellular Infiltrate [NCIt concept]
disease_has_abnormal_cell
- Abnormal Lymphocyte [NCIt concept]
- Malignant Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Lymphocyte [NCIt concept]
- Neoplastic T-Lymphocyte [NCIt concept]
- Neoplastic T-Lymphocyte and Neoplastic Natural Killer Cell [NCIt concept]
disease_has_associated_disease
- Celiac Disease [NCIt concept]
disease_has_finding
- Malignant Cellular Infiltrate [NCIt concept]
- Villous Atrophy [NCIt concept]
disease_has_molecular_abnormality
- Clonal T-Cell Receptor Beta Chain Gene Rearrangement [NCIt concept]
- Clonal T-Cell Receptor Gamma Chain Gene Rearrangement [NCIt concept]
disease_has_normal_cell_origin
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Intraepithelial T-Lymphocyte [NCIt concept]
- Intraepithelial T-Lymphocyte of the Intestine [NCIt concept]
- Lymphocyte [NCIt concept]
- Peripheral (Post-Thymic) T-Lymphocyte and Natural Killer Cell [NCIt concept]
- T-Lymphocyte and Natural Killer Cell [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
- Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Digestive System [NCIt concept]
- Hematopoietic and Lymphatic System [NCIt concept]
- Lymphatic System [NCIt concept]
- Small Intestine [NCIt concept]
disease_may_have_abnormal_cell
- Anaplastic Large T-Lymphocyte [NCIt concept]
- Neoplastic Medium-Sized T-Lymphocyte [NCIt concept]
- Neoplastic Medium-Sized to Large T-Lymphocyte [NCIt concept]
- Pleomorphic Medium-Sized to Large T-Lymphocyte [NCIt concept]
disease_may_have_associated_disease
- EBV Infection [NCIt concept]
- Type II Refractory Celiac Disease [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Gain of Chromosome 9q34 [NCIt concept]
disease_may_have_molecular_abnormality
- JAK1 Gene Mutation [NCIt concept]
- STAT3 Gene Mutation [NCIt concept]

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