Sézary syndrome: Diagnosis, prognosis, and critical review of treatment options

WHO-EORTC classification for cutaneous lymphomas

“…Sézary syndrome (SS) is defined historically by the triad of erythroderma, generalized lymphadenopathy, and the presence of neoplastic T cells (Sézary cells) in skin, lymph nodes, and peripheral blood. 78 In a recent report of the International Society for Cutaneous Lymphomas (ISCL), criteria recommended for the diagnosis of SS include one or more of the following: an absolute Sézary cell count of least 1000 cells/mm 3 ; demonstration of immunophenotypical abnormalities (an expanded CD4 ϩ T-cell population resulting in a CD4/CD8 ratio more than 10, loss of any or all of the T-cell antigens CD2, CD3, CD4, and CD5, or both); or the demonstration of a T-cell clone in the peripheral blood by molecular or cytogenetic methods. 79 It is acknowledged that SS is part of a broader spectrum of erythrodermic CTCL, and that alternative staging systems for assessment of the degree of peripheral blood involvement in these erythrodermic CTCLs have been proposed.…”

“…Lymphadenopathy, alopecia, onychodystrophy, and palmoplantar hyperkeratosis are common findings. 78 Histopathology. The histological features in SS may be similar to those in MF.…”

Activation of Jak/STAT proteins involved in signal transduction pathway mediated by receptor for interleukin 2 in malignant T lymphocytes derived from cutaneous anaplastic large T-cell lymphoma and Sezary syndrome.

“…CTCL has a tendency to progress over time. When it spreads into the blood, the diagnosis of Sezary syndrome (SzS) is made (37)(38)(39). Progression to a high-grade lymphoma (35,36) composed of cells with large nuclei and sometimes anaplastic features also occurs, leading to the diagnosis of the secondary (anaplastic) large T-cell lymphoma (ALCL).…”

Primary Cutaneous Lymphomas and Interferon Treatment

“…Skin-directed therapies like PUVA or potent topical steroids may be used as adjuvant therapy. Extracorporeal photopheresis (ECP), either alone or in combination with other treatment modalities, has been suggested as the treatment option in SS with overall response (OR) rates of 30%-80%, or complete response (CR) rates of 14%-25% [13]. Prolonged treatment with a combination of low-dose chlorambucil and prednisone is often effective in controlling the disease, but is unlikely to give CR.…”