Enteropathy Associated T-Cell Lymphoma: A Clinical Prognostic Model to Identify High Risk Patients

Baaij, Laura R. de; Water, Jolanda M. van de; Verbeek, Wieke H.M.; Visser, Otto; Kuik, Dirk J.; Oudejans, Joost J.; Meijer, Chris J.L.M.; Mulder, Chris J.; Cillessen, Saskia A.G.M.

doi:10.1016/s0016-5085(11)60145-5

Gastroenterology

2011

DOI: 10.1016/s0016-5085(11)60145-5

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Enteropathy Associated T-Cell Lymphoma: A Clinical Prognostic Model to Identify High Risk Patients

Laura R. de Baaij¹,

Jolanda M. van de Water²,

Wieke H.M. Verbeek³

et al.

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“…on April 29, 2019. by guest www.bloodjournal.org From overall survival and failure-free survival in patients with EATL. 34 A preliminary report 35 identified 3 risk factors predictive for survival in a multivariate analysis (ie, raised LDH, presence of Bsymptoms, and secondary EATL). This new prognostic model showed superior predictive capacity compared with the International Prognostic Index and made it possible to identify a high-risk group needing more aggressive therapies.…”

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confidence: 99%

How I treat enteropathy-associated T-cell lymphoma

Sabatino

Biagi

Gobbi

et al. 2012

Blood

104

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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. (Blood. 2012;119(11): 2458-2468) IntroductionCeliac disease (CD) is a chronic gluten-sensitive enteropathy characterized by a high prevalence in the general population and an increased mortality. 1,2 It is well known that the increased mortality is mainly the result of the complications of CD itself, represented by refractory CD (RCD) and enteropathy-associated T-cell lymphoma (EATL). 2 RCD is a form of CD that does not respond histologically to at least 12 months of a strict gluten-free diet (GFD). 3 RCD can also evolve in patients who initially responded normally to a GFD and who are still maintaining a strict GFD. On the basis of the intraepithelial lymphocyte (IEL) population, RCD is further classified into type 1 and type 2. RCD type 1 (RCD1) is characterized by persisting villous atrophy despite a strict GFD associated with increased but still phenotypically normal IELs. Conversely, a clonal expansion of abnormal IELs lacking surface CD3, CD8, and T-cell receptor (TCR) markers but expressing intracellular CD3 indicates RCD type 2 (RCD2), a condition that frequently evolves into EATL, the most serious complication of CD. 4 In the past, up to 20% of celiac patients were considered to be affected by a nonresponsive form of CD. This was obviously because of the lack of consensus on the definition of nonresponsiveness. 4 In the last few years, some evidence has suggested that the prevalence of RCD is low, ranging from 0.6% to 1.5%. 3,5,6 Although the presence of RCD in CD is probably to be approximately 1%, the prevalence of RCD2 among patients with RCD is far from being ascertained. Probably because of not only the great differences in the diagnostic criteria, 4 but also referral bias, different papers report very different results, which vary from 15% to 75%. 7-9 Finally, although the incidence of EATL was reported to be rare in the general population (1 per million person-years), 10,11 it was shown that it occurs in 60% to 80% of patients with RCD2 within 5 years. [7][8][9]12 The description of EATL arising in patients with RCD...

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confidence: 99%

How I treat enteropathy-associated T-cell lymphoma

Sabatino

Biagi

Gobbi

et al. 2012

Blood

104

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Enteropathy Associated T-Cell Lymphoma: A Clinical Prognostic Model to Identify High Risk Patients

Cited by 1 publication

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How I treat enteropathy-associated T-cell lymphoma

How I treat enteropathy-associated T-cell lymphoma

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