Expert Sheds Light on Lesser-Known T-Cell Lymphoma Subtypes

Gina Columbus @ginacolumbusonc
Published Online: Thursday, Jun 22, 2017

Adrienne A. Phillips, MD

Adrienne A. Phillips, MD

Adult T-cell lymphoma/leukemia (ATLL), known as a rarer subtype of peripheral T-cell lymphoma, presents very differently and can be more prevalent in certain worldwide demographics compared with others, explains Adrienne A. Phillips, MD.

“The main take-home message is that this is a rare condition, but if you live in a community where you have immigrants from the Caribbean and Latin America, it is actually not so rare,” said Phillips. “Here, in New York City, we have that population. It is not as uncommon as we are taught in the textbooks. That is the first thing.”

Novel regimens are being explored for these patients. Findings of a multicenter, randomized clinical trial of 71 patients comparing the anti-CCR4 monoclonal antibody mogamulizumab (n = 47) with investigator's choice (n = 24) in the treatment of patients with relapsed/refractory ATLL showed that those who received mogamulizumab had an investigator-assessed overall response rate (ORR) of 34%. The ORR was 23.4% per blinded independent review. The most frequently observed adverse events in the mogamulizumab-treated arm were infusion reactions, rash/drug eruption, and infections.

In an interview, Phillips, an assistant professor at Weill Cornell Medicine/NewYork-Presbyterian Hospital, discussed the subtypes of ATLL, the challenges with treating them, and what community oncologists need to know about these patient populations. Phillips shared this insight during the 2017 OncLive® State of the Science SummitTM on Hematologic Malignancies.

OncLive: Please provide an overview of your presentation on these rarer T-cell lymphomas.

Phillips: ATLL is a rare subtype of peripheral T-cell lymphoma. It has 4 different subtypes: acute, lymphoma, chronic, and smoldering subtypes. It is caused by a virus called HTLV-1, which about 20 million people worldwide are affected with. Most patients are in the Caribbean or Japan; some are in Latin America and Africa. It is relatively uncommon in the United States. Across the nation, approximately less than 1% carry the virus. However, because here in New York City we treat a number of immigrants from the Caribbean and Latin America, we see higher rates. Therefore, we are seeing more ATLL.

Do each of these 4 subtypes have their own treatment strategy, or can you treat them similarly?

That is a good question. Most of the treatment information comes from Japan, where the acute subtype predominates. I have conducted a retrospective series here in New York where we find that our Caribbean patients, even though they might have high white counts and be defined as the acute subtype, also have a lot of adenopathy. In Japan, the treatment for the acute subtype and the lymphoma subtype generally is combination chemotherapy. However, in western countries—because the disease is so rare—the standard is not well defined.

Here, we would treat patients as if they had lymphoma, with combination chemotherapy. Ideally, we would put them on a clinical trial, but I should note that in Europe they conducted a large retrospective meta-analysis that showed, for those with the acute subtype, antiviral therapy with interferon may be useful. We haven’t really found that here in the United States, but again, there are limited patients.

Are there any ongoing studies exploring these populations further?

Absolutely. Although ATLL is a type of T-cell lymphoma, studies of T-cell lymphoma frequently exclude ATLL because those patients have a particularly poor prognosis and the mechanism for their leukemogenesis or lymphomagenesis is related to the virus, which is distinct from some of the other subtypes.




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