This article is more than 5 years old. Information may no longer be current.

Overall survival remained poor for patients with Sézary syndrome

Overall prognoses for patients with Sézary syndrome was reaffirmed to be poor, including a median survival of 4 years after diagnoses, according to the results of a long-term study.

Using a retrospective chart review, researchers studied 176 patients (110 men) who were seen from 1976 to 2010 at the Mayo Clinic in Rochester, Minn., and were identified with Sézary syndrome (SS), a leukemic variant of cutaneous T-cell lymphoma (CTCL). Researchers said the study population represented the largest to date for patients with SS.

The study included patients with T4 (≥80% of body surface area involved with erythroderma) and definitive leukemic involvement. The mean age of the patients at SS diagnosis was 66.2 years, and patients had a median duration of 3 years of dermatologic symptoms before diagnoses (IQR=1.3-6 years).

Many patients underwent lymph node and bone marrow biopsies, and molecular studies of the lymph nodes, bone marrow, blood and skin to verify diagnoses and to exclude other factors.

In a follow-up of all patients, 150 were known to be deceased, with a median time of death after diagnosis of 3.5 years. The median duration of follow-up among the other 26 patients was 4.4 years. After diagnoses, median survival was 4 years; overall survival was 86.1% and 42.3% at 1 and 5 years, respectively. With increasing age, worse prognoses were reported (HR=1.39; 95% CI, 1.20-1.60).

The existence or diagnosis of prior mycosis fungoides (MF), observed in 12 SS patients, also indicated worse prognoses (HR=2.68; 95% CI, 1.44-4.98). Other potential predictors of shorter survival, after adjusting for age, were lactate dehydrogenase level at presentation (HR=1.71; 95% CI, 1.18-2.47 per doubling) and T-cell receptor gene rearrangements in skin (HR=2.59; 95% CI, 1.38-4.87) and blood (HR=2.05; 95% CI, 1.00-4.21).

“Because of the aggressive nature of SS compared with other subsets of CTCL, such as erythrodermic MF or early stage MF, it is imperative to stratify and analyze the distinctive patient population separately,” the researchers reported. “Further studies need to identify improved diagnostic techniques and molecular prognostic markers, and assess the impact of novel therapeutics to improve the outcomes of patients with SS.”