Current Hematologic Malignancy Reports

, Volume 11, Issue 6, pp 504–513 | Cite as

Enteropathy-Associated T-Cell Lymphoma

  • Sarah Ondrejka
  • Deepa JagadeeshEmail author
Part of the following topical collections:
  1. Topical Collection on T-Cell and Other Lymphoproliferative Malignancies


Enteropathy-associated T-cell lymphoma is a rare neoplasm with uniformly aggressive features that arises from intestinal T-cells. There is strong evidence supporting its association as a dire complication of celiac disease. The clinical presentation can vary from malabsorption and abdominal pain to an acute abdominal emergency. Originally, it was divided into types I and II in World Health Organization (WHO) classification schemes, reflective of epidemiology and differences in clinicopathologic features. The debate over the degree of separation of the two types is ongoing as new data emerges regarding the pathogenetics. The low incidence and variable patient factors are major barriers in conducting clinical trials and establishing standard treatment regimens. Yet, the collective experience demonstrates favorable outcomes with combination chemotherapy followed by an autologous hematopoietic stem cell transplant in patients who can tolerate such treatment. The prognosis remains dismal; thus, future research studies are warranted to identify effective novel therapies that can improve outcomes in this rare disease entity.


Intestinal T-cell lymphoma Type I enteropathy-associated T-cell lymphoma Type II enteropathy-associated T-cell lymphoma Monomorphic CD56+ intestinal T-cell lymphoma Celiac disease Refractory celiac disease, monomorphic epitheliotropic intestinal T-cell lymphoma 


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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  1. 1.Pathology and Laboratory Medicine Institute, Cleveland ClinicClevelandUSA
  2. 2.Department of Hematology and Medical OncologyTaussig Cancer Institute, Cleveland ClinicClevelandUSA

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