1. Susman, Ed

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WASHINGTON, DC-The rare cutaneous T-cell lymphoma known as Sezary syndrome appears to have disproportionately poor survival outcomes among the black population when compared with whites, researchers reported at the 74th annual meeting of the American Academy of Dermatology.

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The 5-year overall survival for persons identified as whites in the study was 44.7 percent compared to 15.8 percent survival among blacks (P<0.01), reported Sekwon Jang, MD, a medical oncologist, Inova Melanoma and Skin Cancer Center, Fairfax, Va. He works with Jennifer DeSimone, MD, a dermatologist at Inova in the lymphoma clinic.


"This is the first study which shows that black people with Sezary syndrome have a much poorer survival compared with patients who have white ethnicity," Jang said at his poster discussion sessions. "Five-year relative survival rate which was adjusted for age and gender was poor for white patients but was even worse for black ethnicity," he said.


Jang suggested, "The difference in outcome by racial ethnicity can be explained by several different factors, including disparities in access to care, or by biological aggressiveness of the disease, or delayed diagnosis because the disease is much more difficult to diagnose in patients with darker skin compared with patients with fairer skin. There may also be some differences in treatment efficacy between racial groups.


"Our study showed poorer survival in black patients with Sezary syndrome," he said. "Obviously, we need more research to understand the main differences and how we can overcome these racial disparities where they exist."


In commenting on the study, Katy Burris, MD, a dermatologist at Northwell Health, Manhasset, N.Y., told Oncology Times, "This study is unique in that it suggests that black patients with Sezary syndrome have poorer 5-year survival rates when compared to white patients. However, only 11 percent of the patients were black, which is a relatively small number, which may make it difficult to apply this to the cutaneous T-cell lymphoma population as a whole."


She concurred the disparity could be due to issues in access to care between the two populations, which could cause "delay in diagnosis or limited access to certain medications or treatments may ultimately lead to a poorer outcome. We also don't know from this study if the cancer behaves more aggressively in certain populations due to differences in biology. More studies are needed to explore these questions," Burris said.


Sezary Syndrome Statistics

According to the Cutaneous Lymphoma Foundation, the two most common types of cutaneous T-cell lymphomas are mycosis fungoides, which is often indolent, appears as reddish skin patches and can progress over many years, and an advanced and leukemic form of mycosis fungoides called Sezary syndrome.


Sezary syndrome is distinguished from mycosis fungoides by the presence of malignant lymphocytes in the blood and is characterized by extensive thin red, itchy rashes covering over 80 percent of the body. In some cases, thicker, red plaques and tumors may also appear. In addition, these symptoms may be accompanied by changes in the nails, hair or eyelids or the presence of enlarged lymph nodes.


The foundation estimates 3,000 new cases of mycosis fungoides occur each year in the U.S. and approximately 15 percent of those are diagnosed as Sezary syndrome. Although this type of non-Hodgkin lymphoma can affect people of any age, Sezary syndrome usually occurs in adults ages 50 and over and is slightly more common in men than women. There are no known risk factors for this type of cutaneous T-cell lymphoma.


In presenting the study, Jang said he and colleagues analyzed patients with Sezary syndrome from 1973-2012 using data from Surveillance, Epidemiology, and End Results (SEER)*Stat - Version 8.2.1. The database covers about 20 percent of the U.S. population, he said.


"There is really limited data on the racial difference in survival of patients with Sezary syndrome," Jang said. "Previous publications have included other conditions such as mycosis fungoides with Sezary syndrome so it has been difficult to differentiate data that focuses on patients with just Sezary syndrome. The diagnosis of Sezary syndrome carries a very poor prognosis," he said, noting the outcomes in his study.


* Survival at one year was 82 percent among whites and 69.9 percent among blacks.


* At 2 years survival was 69.2 percent among whites and 31 percent among blacks.


* At 3 years survival was 61.7 percent among whites and 15.8 percent among blacks.


* At 4 years survival was 50.6 percent among whites and remained at 15.8 percent among blacks.


* At 5 years survival was 44.7 percent among whites and was still 15.8 percent among blacks.



"Over the period of about 40 years, we found 204 patients with Sezary syndrome," Jang said. "So you can see that this is a very rare diagnosis. It is also believed that in the 1970s and 1980s there was likely misdiagnosis of Sezary syndrome."


Sixty-four percent of patients in the study were male; the median age at diagnosis was 68. About 83 percent of the patients identified as white; about 11 percent were black and 6 percent were classified as other, which included Asians, Jang said. No significant difference in relative survival was found between whites and "others."


Of the 169 white patients in the study, 71 were younger than 65 years-42 percent; 13 of the 22 black patients were younger than 65 when diagnosed; seven of the 12 persons of other races were younger than 65, the researchers reported.


About 63 percent or 107 patients who were white were men; 64 percent of the blacks were men and men made up 75 percent of people of other races.


Ed Susman is a contributing writer.