How I treat enteropathy-associated T-cell lymphoma

Blood ◽  
2012 ◽  
Vol 119 (11) ◽  
pp. 2458-2468 ◽  
Author(s):  
Antonio Di Sabatino ◽  
Federico Biagi ◽  
Paolo G. Gobbi ◽  
Gino R. Corazza
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
High Dose Chemotherapy ◽  
T Cell Lymphoma ◽  
Gluten Free Diet ◽  
High Dose ◽  
Gluten Free ◽  
Strict Adherence ◽  
Positron Emission ◽  
Magnetic Resonance Enteroclysis

Abstract 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.

Are Complicated Forms of Celiac Disease Cryptic T-Cell Lymphomas?

Blood ◽  
1998 ◽  
Vol 92 (10) ◽  
pp. 3879-3886 ◽  
Author(s):  
Franck Carbonnel ◽  
Laurence Grollet-Bioul ◽  
Jean Claude Brouet ◽  
Marie Françoise Teilhac ◽  
Jacques Cosnes ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Celiac Disease ◽  
T Cell ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Gluten Free Diet ◽  
Gluten Free ◽  
Duodenal Biopsies

Abstract We assessed the clonality of duodenal mucosal T cells in patients with celiac disease and controls. Fifteen adult patients were studied. Four patients had a complicated celiac disease, 3 did not respond to a gluten-free diet, and 2 had an ulcerative jejunitis (including 1 patient with nonresponsive celiac disease). Seven patients had an untreated celiac disease responsive to a gluten-free diet. Histological examination of duodenal biopsies of these 11 patients showed benign-appearing celiac disease without evidence of lymphoma. Four patients with nonulcer dyspepsia and normal duodenal biopsies served as controls. TCRγ gene rearrangements were analyzed by multiplex polymerase chain reaction on DNA extracted from duodenal biopsies. Major clonal rearrangements of the T-cell receptor were found in 4 cases, all with complicated celiac disease. Monoclonality was confirmed by DNA sequencing of the junctional region in 3 cases and by hybridization with clone-specific oligoprobes. Patients with celiac disease responsive to gluten-free diet had mainly a polyclonal pattern, with 1 of them having an oligoclonal rearrangement. An oligoclonal pattern was also observed in 2 control patients. Three patients with complicated celiac disease evolved to T-cell lymphoma with liver (n = 2) or bone marrow (n = 1) invasion. Identical clones were found in the enteropathic duodenojejunum and peripheral blood in the patient with large-cell lymphoma with bone marrow invasion. This study suggests that complicated celiac disease is a cryptic T-cell lymphoma.

Are Complicated Forms of Celiac Disease Cryptic T-Cell Lymphomas?

Blood ◽  
1998 ◽  
Vol 92 (10) ◽  
pp. 3879-3886
Author(s):  
Franck Carbonnel ◽  
Laurence Grollet-Bioul ◽  
Jean Claude Brouet ◽  
Marie Françoise Teilhac ◽  
Jacques Cosnes ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Celiac Disease ◽  
T Cell ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Gluten Free Diet ◽  
Gluten Free ◽  
Duodenal Biopsies

We assessed the clonality of duodenal mucosal T cells in patients with celiac disease and controls. Fifteen adult patients were studied. Four patients had a complicated celiac disease, 3 did not respond to a gluten-free diet, and 2 had an ulcerative jejunitis (including 1 patient with nonresponsive celiac disease). Seven patients had an untreated celiac disease responsive to a gluten-free diet. Histological examination of duodenal biopsies of these 11 patients showed benign-appearing celiac disease without evidence of lymphoma. Four patients with nonulcer dyspepsia and normal duodenal biopsies served as controls. TCRγ gene rearrangements were analyzed by multiplex polymerase chain reaction on DNA extracted from duodenal biopsies. Major clonal rearrangements of the T-cell receptor were found in 4 cases, all with complicated celiac disease. Monoclonality was confirmed by DNA sequencing of the junctional region in 3 cases and by hybridization with clone-specific oligoprobes. Patients with celiac disease responsive to gluten-free diet had mainly a polyclonal pattern, with 1 of them having an oligoclonal rearrangement. An oligoclonal pattern was also observed in 2 control patients. Three patients with complicated celiac disease evolved to T-cell lymphoma with liver (n = 2) or bone marrow (n = 1) invasion. Identical clones were found in the enteropathic duodenojejunum and peripheral blood in the patient with large-cell lymphoma with bone marrow invasion. This study suggests that complicated celiac disease is a cryptic T-cell lymphoma.

Alternating non-cross-resistant multiagent chemotherapy (ANCRC) and high-dose chemotherapy followed by autologous stem cell support (HDC-ASCS) in first remission to improve outcome in patients with T-cell lymphoma.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e18538-e18538
Author(s):  
Potjana Jitawatanarat ◽  
Richa Dawar ◽  
Kaweesak Chittawatanarat ◽  
Nicolas Batty ◽  
Myron Stefan Czuczman ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Final Analysis ◽  
High Dose Chemotherapy ◽  
T Cell Lymphoma ◽  
High Dose ◽  
Front Line ◽  
First Remission ◽  
The Impact

e18538 Background: T-cell lymphomas represent a challenge for the practicing oncologist as evidence-based medicine is limited and the ORR to chemotherapy, PFS and OS rates are inferior when compared to B-cell lymphoma. CHOP offers limited activity, and there is a growing consensus that alternative regimens should be tested. We studied the impact of ANCRC or HDC-ASCS vs. CHOP in outcome of T-cell lymphoma. Methods: We retrospectively analyzed differences between the ORR, PFS and OS of T-cell lymphoma patients excluding ALCL treated with CHOP or HyperCVAD regimen alternating with HDAM in the front-line setting following by observation or HDC-ASCS. Pre-treatment demographic, clinical, and pathological characteristics were collected. Differences in outcomes were analyzed using a Cox proportional analysis. Results: ALCL, PTCL-NOS and AITL were the most common subtypes. After excluding ALCL (N=29), a total of 49 patients were included in the final analysis; 23 pts treated with CHOP; 12 pts with HyperCVAD/HDAM and 14 pts treated with other induction regimens. After induction therapy, 12 patients underwent HDC-ASCS in first remission. There were no differences in demographic and clinical characteristics between groups analyzed. There was a higher ORR and PFS among patients treated with hyperCVAD/HDAM (83.3%) vs. pts treated with CHOP (62%). However it did not improved OS when compared to CHOP. The use of HDC-ASCS in first remission was associated with improved OS. The median OS for pts observed after front-line chemotherapy was 32 months and 59 months for pts that received HDC-ASCS [Hazard ratio 0.54 (95% CI 0.16-1.83) (p=0.32)]. Conclusions: Our data suggest that hyperCVAD/HDAM results in higher ORR and PFS in T-cell lymphoma than CHOP. More importantly, the use of HDC-ASCS in first remission appears to improve the OS of non-ALCL T-cell lymphoma patients. ANCRC regimens incorporating novel agents follow by HDC-ASCS in first remission is emerging as an attractive therapeutic intervention for a selected group of T-cell lymphoma patients been evaluated in ongoing clinical trials.

Effect of a Gluten-free Diet on the Risk of Enteropathy-associated T-cell Lymphoma in Celiac Disease

2007 ◽  
Vol 53 (4) ◽  
pp. 972-976 ◽  
Author(s):  
Marco Silano ◽  
Umberto Volta ◽  
Alessandro De Vincenzi ◽  
Mariarita Dessì ◽  
Massimo De Vincenzi ◽  
...  
Keyword(s):  
Celiac Disease ◽  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Gluten Free Diet ◽  
Gluten Free
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