Enteropathy-associated T-cell lymphoma (type II): a Brazilian case report
Autopsy And Case Reports
Enteropathy-associated T-cell lymphoma (type II): a Brazilian case report
Autor Correspondente: A. Felipe-Silva | [email protected]
Palavras-chave: enteropathy-associated T-cell lymphoma; celiac disease; lymphoma, non-hodgkin; intestinal perforation; lymphoma, T-cell.
Resumos Cadastrados
Resumo Inglês:
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.
