Enteropathy Associated T Cell Lymphoma C0456889
A rare, aggressive T-cell lymphoma of the intestines linked to celiac disease. It primarily affects adults, often those with long-standing gluten sensitivity. Treatment includes chemotherapy, though prognosis remains challenging.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Abdominal pain
Intestinal obstruction
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Weight loss
(oncologic)Diarrhea
(oncologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Intensive Combination Chemotherapy
Multi-agent chemotherapy regimen typically including anthracycline-based protocols such as CHOP or enhanced regimens like SMILE for enteropathy-associated T-cell lymphoma. Given the aggressive nature of this disease, intensive treatment is essential.
Gluten-Free Diet and Nutritional Support
Strict gluten avoidance remains important even after lymphoma diagnosis, as continued gluten exposure may exacerbate intestinal inflammation. Comprehensive nutritional support addresses the protein-losing enteropathy and malabsorption common in these patients.
Additional treatment options
Autologous Stem Cell Transplantation
High-dose chemotherapy followed by rescue with harvested stem cells, offered to eligible patients achieving remission. This consolidates treatment response and may improve long-term disease control.
procedureSurgical Management of Complications
Emergency or planned surgery for intestinal perforation, obstruction, or hemorrhage that may occur as presenting features or complications of enteropathy-associated T-cell lymphoma. Includes bowel resection and stoma creation as needed.
surgicalPsychological and Supportive Care
Mental health support addressing the significant psychological burden of this rare diagnosis combined with celiac disease management. Includes counseling, support groups, and help with coping strategies for chronic illness management.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Enteropathy Associated T Cell Lymphoma C0456889?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Enteropathy Associated T Cell Lymphoma C0456889. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy