Enteropathy-associated T-cell lymphoma (type II): a Brazilian case report

  • Aloísio Felipe-Silva Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Maria Claudia Nogueira Zerbini Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Fernando Peixoto Ferraz de Campos Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Raphael Salles Scortegagna de Medeiros Instituto do Câncer do Estado de São Paulo, São Paulo/SP - Brazil.
  • Rodrigo Montenegro Lourenção Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Keywords: Enteropathy-Associated T-Cell Lymphoma, Celiac Disease, Lymphoma, Non-Hodgkin, Intestinal Perforation, T-Cell.

Abstract

Enteropathy-associated T-cell Lymphoma (EATL) is a rare form of aggressive T-cell lymphoma. It is more prevalent in men over 60 years and the prognosis is very poor. EATL is classified into two groups based on morphology, immunohistochemistry, and genetic profile. EATL type I is highly associated with celiac disease and is more common in Western countries. EATL type II predominates over type I in Asia, where celiac disease is uncommon. We report a case of a 78-year-old previously healthy white male who presented with a 2-month history of diarrhea, weight loss and edema. The abdomen was distended
and painful, and a tumor mass was palpable in the hypogastrium. Laboratory tests showed hypoalbuminemia. Serological tests for HIV, viral hepatitis and HTLV-1 were negative. The chest radiography showed pneumoperitoneum,
and an exploratory laparotomy revealed perforation of the small bowel. An advanced stage (Ann Arbor IV B/Lugano IIE2B) EATL type II was diagnosed.
Four cycles of chemotherapy were interspersed with several complications (anthracycline-induced cardiotoxicity, chemotherapy-induced neutropenic fever and severe sepsis). Performance status progressively worsened and he died 6 months after the diagnosis. This is an illustrative report of a rare and aggressive primary intestinal lymphoma. To the best of our knowledge, this is the first report of EATL type II in Brazil.

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Author Biographies

Aloísio Felipe-Silva, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Maria Claudia Nogueira Zerbini, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.

Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.

Department of Pathology - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.

Fernando Peixoto Ferraz de Campos, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Department of Internal Medicine - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Raphael Salles Scortegagna de Medeiros, Instituto do Câncer do Estado de São Paulo, São Paulo/SP - Brazil.
Instituto do Câncer do Estado de São Paulo, São Paulo/SP - Brazil.
Rodrigo Montenegro Lourenção, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Department of Surgery - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Published
2012-06-08
How to Cite
Felipe-Silva, A., Zerbini, M., de Campos, F., de Medeiros, R., & Lourenção, R. (2012). Enteropathy-associated T-cell lymphoma (type II): a Brazilian case report. Autopsy and Case Reports, 2(2). https://doi.org/10.4322/acr.%y.36460
Section
Article / Clinical Case Report