Alternative type of enteropathy-associated T-cell lymphoma is identified

An international research team has identified a new type of deadly intestinal lymphoma that is particularly common in Asia. The team also developed a new diagnostic test to accurately identify the disease.

The study, carried out by the Singapore Lymphoma Study Group at Singapore General Hospital (SGH) and the National Cancer Centre Singapore (NCCS), has an immediate impact on patient care. Doctors can diagnose this lymphoma type accurately and tailor more effective treatment strategies to improve patient outcomes. It will also impact the most recent World Health Organization (WHO) classification of hematolymphoid neoplasms.

This is the largest study of this lymphoma type, involving 60 cases from centers in Singapore and around Asia, including South Korea, Hong Kong, Taiwan, Australia, China, and Malaysia. The findings were published in Leukemia (2013; doi:10.1038/leu.2013.41 ).

The disease, almost unheard of before 2008, has been classified as an alternative type of enteropathy-associated T-cell lymphoma (EATL Type I), a disease common in Caucasians and associated with celiac disease.

“We discovered that the intestinal lymphoma commonly seen in Asian patients has no links to celiac disease or EATL Type I found in Caucasians,” said first author Tan Soo Yong, MD, of the Department of Pathology at SGH and faculty with Duke-NUS. “Instead, we discovered that the pathology of this disease is very different and most likely originates from a unique epithelial cell type found in the intestine, making it a completely different disease type.”

“We, therefore, propose to reclassify the disease, currently labeled EATL Type II, as ‘Epitheliotropic Intestinal T-cell Lymphoma' (EITL),” added Tan. This would impact the WHO classification.

In addition, the team has identified a novel biomarker, known as MATK (megakaryocyte-associated tyrosine kinase), and developed a diagnostic test that enables clinicians to accurately diagnose this type of lymphoma. Requests for this test have come in from around the world, including China and the United States.

“Our research has an immediate impact on the care we can provide to patients with this rare but very aggressive intestinal lymphoma,” said Lim Soon Thye, MD, of the Department of Medical Oncology at NCCS and associate professor at Duke-NUS.  “With an accurate diagnosis, we can treat our patients better and improve overall survival.” The average overall survival observed by the researchers was only 7 months. 

Next, the researchers plan to collaborate with international experts in the United States and Canada to investigate the cell of origin and explore immunological approaches to block its growth.

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