Sézary Syndrome and Related Variants of Classic Cutaneous T-cell Lymphoma. A Descriptive and Prognostic Clinicopathologic Study of 29 Cases*

Abstract

Large series of patients with Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL), have been reported infrequently because of its low incidence. Here we recorded several clinical, histopathological and immunophenotypical features of 29 cases of leukemic CTCL patients from four Dermatology Departments of Catalonia, Spain, and analyzed their prognostic value. Clinical data included sex, age, delay of SS diagnosis, previous diagnosis of lymphoma, B-symptoms, type of skin lesions, peripheral adenopathy, histologic evaluation of lymph node biopsy, visceral involvement, percentage of circulating Sézary cells, serum LDH and beta-2-microglobulin levels, first treatment and response, disease-free interval, further therapies and survival. Histopathological data examined were epidermotropism, depth and thickness of the infiltrate, cell size, adnexal involvement, presence of granuloma, eosinophils and plasma cells, mitotic rate. The percentage of CD45Ro, CD43, CD20, CD30 and CD8 positive dermal cells were also recorded. Survival showed a mean actuarial risk of 57% at 3 years and 38% at 5 years, with a median survival of 48 months. Analysis of actuarial survival demonstrated as following as features linked with a bad prognosis: fast evolution of the disease (from symptoms onset up to diagnosis) (p = 0.0274) raised levels of serum lactate dehydrogenase (p = 0.0379) and beta-2-microglobulin (p = 0.0151), the latter being the most important prognostic factor. In conclusion although SS had been traditionally considered as a low-grade lymphoma, the present study agrees with the recent classification rating SS as an aggressive type of CTCL with a poor prognosis. Our results show that some simple clinical and blood test data can be useful as prognostic indicators in this disease.

Expression of T-plastin, FoxP3 and other tumor-associated markers by leukemic T-cells of cutaneous T-cell lymphoma

“…The poor prognosis of Sézary patients whose circulating neoplastic T-cells express FoxP3 transcripts is a new finding that needs to be validated on fresh samples from a larger series of patients. If confirmed, then FoxP3 expression by neoplastic cells can be added to the list of other prognostic indicators for SS such as absolute Sézary cell count [46,47], serum level of lactate dehydrogenase [94,95] or soluble interleukin-2 receptor [96,97], and gene expression profile [37]. The gene expression profile of T-plastin, FoxP3 and other markers of T-cell activation in peripheral blood mononuclear cell samples from patients with Sézary syndrome compared with normal controls.…”

The role of 9-O-acetylated ganglioside D3 (CD60) and 4 1 (CD49d) expression in predicting the survival of patients with Sezary syndrome

“…[13][14][15][16][17][18][19][20] However, none of these markers is directly associated with the tumor phenotype of the SS cells and they seem to be mere markers of risk or, at best, the result of some deregulated mechanisms. Here we describe, for the first time, the prognostic significance of two antigens directly related to T-cell function, and thus possibly involved in the pathogenesis of SS.…”

“…4,5,11,12 Previous studies of prognostic indicators in SS showed that circulating Sézary cell count, high CD4/CD8 ratio, advanced age, high lactate dehydrogenase serum level and a high white blood cell count were associated with an unfavorable outcome. [13][14][15][16][17][18][19][20] Up to now, scanty data have been provided concerning possible associations between the immunophenotype of circulating CD4 + T cells and survival in SS. As for other hematologic malignancies, 21,22 it is important to clarify the prognostic relevance of activation of homing receptors expressed by circulating CD4 + T cells in SS, since these markers might be directly linked to the pathogenesis and outcome of the disorder.…”

Two Young Men with Leonine Facies and Generalized Itching - Two Case Reports of Sezary Syndrome

“…Mycosis fungoides behaves in a manner similar to other low-grade or indolent non-Hodgkin lymphomas. But patients with Sézary syndrome have a relatively poor prognosis, with a median survival of ∼3 to 4 years [8]. Infection remains the most common cause of death in patients with CTCL, with Staphylococcus aureus and Pseudomonas aeruginosa being the most common pathogens infecting the skin, leading to bacteremia and sepsis [9].…”