How I treat enteropathy-associated T-cell lymphoma

Enteropathy-associated T-cell lymphoma (EATL) is a complication of celiac disease (CD). This tumor derives from the neoplastic transformation of aberrant intraepithelial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozy...

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Bibliographic Details
Published in:Blood 2012-03, Vol.119 (11), p.2458-2468
Main Authors: Di Sabatino, Antonio, Biagi, Federico, Gobbi, Paolo G., Corazza, Gino R.
Format: Article
Language:English
Subjects:
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Biological and medical sciences
Celiac Disease - etiology
Celiac Disease - therapy
Combined Modality Therapy
Diet, Gluten-Free
Enteropathy-Associated T-Cell Lymphoma - complications
Enteropathy-Associated T-Cell Lymphoma - diagnosis
Enteropathy-Associated T-Cell Lymphoma - therapy
Hematologic and hematopoietic diseases
Hematopoietic Stem Cell Transplantation
HLA-DQ Antigens - metabolism
Homozygote
Humans
Medical sciences
Middle Aged
Prognosis
Radiotherapy Dosage
Risk Factors
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Description
Summary:Enteropathy-associated T-cell lymphoma (EATL) is a complication of celiac disease (CD). This tumor derives from the neoplastic transformation of aberrant intraepithelial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozygosity, and late diagnosis of CD are recognized as risk factors for malignant evolution of CD. Recurrence of diarrhea, unexplained weight loss, abdominal pain, fever, and night sweating should alert physicians to this complication. The suspicion of EATL should lead to an extensive diagnostic workup in which magnetic resonance enteroclysis, positron emission tomography scan, and histologic identification of lesions represent the best options. Treatment includes high-dose chemotherapy preceded by surgical resection and followed by autologous stem cell transplantation, although biologic therapies seem to be promising. Strict adherence to a gluten-free diet remains the only way to prevent EATL.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2011-10-385559