Enteropathy-associated T-cell lymphoma (patient information)
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Enteropathy-associated T-cell lymphoma |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2], Sowminya Arikapudi, M.B,B.S. [3]
Overview
- Enteropathy-associated T cell lymphoma is a very rare but aggressive form of lymphoma, it has a strong association with celiac disease and very poor prognosis.
- There are two subtypes of the tumor, type I is more common in European origin patients, type II is most common among the Asian population. Patients can have different symptoms such as night sweat, weight loss, fever, diarrhea, swollen lymph nodes.
What are the symptoms of Enteropathy-associated T-cell lymphoma?
Some of the symptoms of EATL include:[1]
- Abdominal pain
- Diarrhea
- Unexpected weight loss
- Skin lesion
- Fever
- Night sweat
What causes Enteropathy-associated T-cell lymphoma?
There aren't sufficient studies regarding causes of Enteropathy-associated T-cell lymphoma, in other words, it's not known causes for this disease.
Who is at highest risk?
- Enteropathy-associated T cell lymphoma type I is more common among those diagnosed with celiac disease, however, type II can be seen in those without celiac disease
- There is a male gender predilection.
Diagnosis
- It is recommended that patients coming with malabsorption symptoms such as diarrhea, unintentional weight loss, or painless swollen lymph nodes, night sweats, fever should be considered for the diagnosis of gastrointestinal lymphoma, extensive workup must be done such as complete blood count, imaging studies such as abdominal CT scan, PET scan but final diagnosis needs endoscopy and biopsy of the specimen.[2]
When to seek urgent medical care?
- Patients who suffer from malabsorption, unintentional weight loss, diarrhea, fever, night sweat must visit their primary care physicians in order to have an extensive workup done and rule out intestinal lymphoma.
Treatment options
- Unfortunately tumor is very aggressive in nature and mainstay therapy is supportive. Chemotherapy, surgery or stem cell transplant can be part of treatment depending on the tumor progression and metastasis to adjacent lymph nodes or other internal organs.
Where to find medical care for Enteropathy-associated T-cell lymphoma?
- Contact your medical provider if you experience symptoms such as diarrhea, unintentional weight loss, nausea/vomiting, fever, night sweat, painless swollen nodes.
- Various treatment regimens are available, depending on the tumor progression treatment options are different. Unfortunately, most patients at the time of diagnosis need surgery and debulking.
- Medical care for enteropathy-associated T-cell lymphoma can be found here.
Prevention of Enteropathy-associated T-cell lymphoma
- There is no prevention methods for enteropathy-associated T cell lymphoma.
What to expect (Outlook/Prognosis)?
- Prognosis is very poor and the tumor is very aggressive in nature.
Possible complications
- some of the possible complications of enteropathy-associated T cell lymphoma include:
- Small bowel obstruction
- Perforation
- Intussusception
Source
- ↑ Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES (May 2011). "The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications". Blood. 117 (19): 5019–32. doi:10.1182/blood-2011-01-293050. PMC 3109529. PMID 21300984.
- ↑ Uematsu N, Sumi M, Kaiume H, Takeda W, Kirihara T, Ueki T, Hiroshima Y, Ueno M, Ichikawa N, Watanabe M, Kobayashi H (2018). "[Neurolymphomatosis due to enteropathy-associated T-cell lymphoma clinically diagnosed by FDG-PET/CT and subsequently confirmed by autopsy]". Rinsho Ketsueki (in Japanese). 59 (1): 69–74. doi:10.11406/rinketsu.59.69. PMID 29415941.