Enteropathy-associated T-cell lymphoma: Clinical and histological findings from the international peripheral T-Cell lymphoma project

Jan Delabie, Harald Holte, Julie Marie Vose, Fred Ullrich, Elaine S. Jaffe, Kerry J. Savage, Joseph M. Connors, Lisa Rimsza, Nancy L. Harris, Konrad Müller-Hermelink, Thomas Rüdiger, Bertrand Coiffier, Randy D. Gascoyne, Françoise Berger, Kensei Tobinai, Wing Y. Au, Raymond Liang, Emili Montserrat, Ephraim P. Hochberg, Stefano PileriMassimo Federico, Bharat Nathwani, James Olen Armitage, Dennis D. Weisenburger

Research output: Contribution to journalArticle

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Abstract

Few large, international series of enteropathy-associated T-cell lymphoma (EATL) have been reported. We studied a cohort of 62 patients with EATL among 1153 patients with peripheral T-cell or natural killer (NK) - cell lymphoma from 22 centers worldwide. The diagnosis was made by a consensus panel of 4 expert hematopathologists using World Health Organization (WHO) criteria. Clinical correlations and survival analyses were performed. EATL comprised 5.4% of all lymphomas in the study and was most common in Europe (9.1%), followed by North America (5.8%) and Asia (1.9%). EATL type 1 was more common (66%) than type 2 (34%), and was especially frequent in Europe (79%). A clinical diagnosis of celiac sprue was made in 32.2% of the patients and was associated with both EATL type 1 and type 2. The median overall survival was only 10 months, and the median failure-free survival was only 6 months. The International Prognostic Index (IPI) was not as good a predictor of survival as the Prognostic Index for Peripheral T-Cell Lymphoma (PIT). Clinical sprue predicted for adverse survival independently of the PIT. Neither EATL subtype nor other biologic parameters accurately predicted survival. Our study confirms the poor prognosis of patients with EATL and the need for improved treatment options.

Original languageEnglish (US)
Pages (from-to)148-155
Number of pages8
JournalBlood
Volume118
Issue number1
DOIs
StatePublished - Jul 7 2011

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Enteropathy-Associated T-Cell Lymphoma
Peripheral T-Cell Lymphoma
T-cells
Survival
Celiac Disease
Lymphoma
Northern Asia
Survival Analysis
North America
Natural Killer Cells
T-Lymphocytes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Enteropathy-associated T-cell lymphoma : Clinical and histological findings from the international peripheral T-Cell lymphoma project. / Delabie, Jan; Holte, Harald; Vose, Julie Marie; Ullrich, Fred; Jaffe, Elaine S.; Savage, Kerry J.; Connors, Joseph M.; Rimsza, Lisa; Harris, Nancy L.; Müller-Hermelink, Konrad; Rüdiger, Thomas; Coiffier, Bertrand; Gascoyne, Randy D.; Berger, Françoise; Tobinai, Kensei; Au, Wing Y.; Liang, Raymond; Montserrat, Emili; Hochberg, Ephraim P.; Pileri, Stefano; Federico, Massimo; Nathwani, Bharat; Armitage, James Olen; Weisenburger, Dennis D.

In: Blood, Vol. 118, No. 1, 07.07.2011, p. 148-155.

Research output: Contribution to journalArticle

Delabie, J, Holte, H, Vose, JM, Ullrich, F, Jaffe, ES, Savage, KJ, Connors, JM, Rimsza, L, Harris, NL, Müller-Hermelink, K, Rüdiger, T, Coiffier, B, Gascoyne, RD, Berger, F, Tobinai, K, Au, WY, Liang, R, Montserrat, E, Hochberg, EP, Pileri, S, Federico, M, Nathwani, B, Armitage, JO & Weisenburger, DD 2011, 'Enteropathy-associated T-cell lymphoma: Clinical and histological findings from the international peripheral T-Cell lymphoma project', Blood, vol. 118, no. 1, pp. 148-155. https://doi.org/10.1182/blood-2011-02-335216
Delabie, Jan ; Holte, Harald ; Vose, Julie Marie ; Ullrich, Fred ; Jaffe, Elaine S. ; Savage, Kerry J. ; Connors, Joseph M. ; Rimsza, Lisa ; Harris, Nancy L. ; Müller-Hermelink, Konrad ; Rüdiger, Thomas ; Coiffier, Bertrand ; Gascoyne, Randy D. ; Berger, Françoise ; Tobinai, Kensei ; Au, Wing Y. ; Liang, Raymond ; Montserrat, Emili ; Hochberg, Ephraim P. ; Pileri, Stefano ; Federico, Massimo ; Nathwani, Bharat ; Armitage, James Olen ; Weisenburger, Dennis D. / Enteropathy-associated T-cell lymphoma : Clinical and histological findings from the international peripheral T-Cell lymphoma project. In: Blood. 2011 ; Vol. 118, No. 1. pp. 148-155.
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abstract = "Few large, international series of enteropathy-associated T-cell lymphoma (EATL) have been reported. We studied a cohort of 62 patients with EATL among 1153 patients with peripheral T-cell or natural killer (NK) - cell lymphoma from 22 centers worldwide. The diagnosis was made by a consensus panel of 4 expert hematopathologists using World Health Organization (WHO) criteria. Clinical correlations and survival analyses were performed. EATL comprised 5.4{\%} of all lymphomas in the study and was most common in Europe (9.1{\%}), followed by North America (5.8{\%}) and Asia (1.9{\%}). EATL type 1 was more common (66{\%}) than type 2 (34{\%}), and was especially frequent in Europe (79{\%}). A clinical diagnosis of celiac sprue was made in 32.2{\%} of the patients and was associated with both EATL type 1 and type 2. The median overall survival was only 10 months, and the median failure-free survival was only 6 months. The International Prognostic Index (IPI) was not as good a predictor of survival as the Prognostic Index for Peripheral T-Cell Lymphoma (PIT). Clinical sprue predicted for adverse survival independently of the PIT. Neither EATL subtype nor other biologic parameters accurately predicted survival. Our study confirms the poor prognosis of patients with EATL and the need for improved treatment options.",
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AU - Delabie, Jan

AU - Holte, Harald

AU - Vose, Julie Marie

AU - Ullrich, Fred

AU - Jaffe, Elaine S.

AU - Savage, Kerry J.

AU - Connors, Joseph M.

AU - Rimsza, Lisa

AU - Harris, Nancy L.

AU - Müller-Hermelink, Konrad

AU - Rüdiger, Thomas

AU - Coiffier, Bertrand

AU - Gascoyne, Randy D.

AU - Berger, Françoise

AU - Tobinai, Kensei

AU - Au, Wing Y.

AU - Liang, Raymond

AU - Montserrat, Emili

AU - Hochberg, Ephraim P.

AU - Pileri, Stefano

AU - Federico, Massimo

AU - Nathwani, Bharat

AU - Armitage, James Olen

AU - Weisenburger, Dennis D.

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N2 - Few large, international series of enteropathy-associated T-cell lymphoma (EATL) have been reported. We studied a cohort of 62 patients with EATL among 1153 patients with peripheral T-cell or natural killer (NK) - cell lymphoma from 22 centers worldwide. The diagnosis was made by a consensus panel of 4 expert hematopathologists using World Health Organization (WHO) criteria. Clinical correlations and survival analyses were performed. EATL comprised 5.4% of all lymphomas in the study and was most common in Europe (9.1%), followed by North America (5.8%) and Asia (1.9%). EATL type 1 was more common (66%) than type 2 (34%), and was especially frequent in Europe (79%). A clinical diagnosis of celiac sprue was made in 32.2% of the patients and was associated with both EATL type 1 and type 2. The median overall survival was only 10 months, and the median failure-free survival was only 6 months. The International Prognostic Index (IPI) was not as good a predictor of survival as the Prognostic Index for Peripheral T-Cell Lymphoma (PIT). Clinical sprue predicted for adverse survival independently of the PIT. Neither EATL subtype nor other biologic parameters accurately predicted survival. Our study confirms the poor prognosis of patients with EATL and the need for improved treatment options.

AB - Few large, international series of enteropathy-associated T-cell lymphoma (EATL) have been reported. We studied a cohort of 62 patients with EATL among 1153 patients with peripheral T-cell or natural killer (NK) - cell lymphoma from 22 centers worldwide. The diagnosis was made by a consensus panel of 4 expert hematopathologists using World Health Organization (WHO) criteria. Clinical correlations and survival analyses were performed. EATL comprised 5.4% of all lymphomas in the study and was most common in Europe (9.1%), followed by North America (5.8%) and Asia (1.9%). EATL type 1 was more common (66%) than type 2 (34%), and was especially frequent in Europe (79%). A clinical diagnosis of celiac sprue was made in 32.2% of the patients and was associated with both EATL type 1 and type 2. The median overall survival was only 10 months, and the median failure-free survival was only 6 months. The International Prognostic Index (IPI) was not as good a predictor of survival as the Prognostic Index for Peripheral T-Cell Lymphoma (PIT). Clinical sprue predicted for adverse survival independently of the PIT. Neither EATL subtype nor other biologic parameters accurately predicted survival. Our study confirms the poor prognosis of patients with EATL and the need for improved treatment options.

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